SBT-272 improves TDP-43 pathology in ALS upper motor neurons by modulating mitochondrial integrity, motility, and function
Mitochondrial defects are one of the common underlying causes of neuronal vulnerability in neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), and TDP-43 pathology is the most commonly observed proteinopathy. Disrupted inner mitochondrial membrane (IMM) reported in the upper mot...
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| Vydáno v: | Neurobiology of disease Ročník 178; s. 106022 |
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| Hlavní autoři: | , , , , , , , , , , , , , , |
| Médium: | Journal Article |
| Jazyk: | angličtina |
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United States
Elsevier Inc
01.03.2023
Elsevier |
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| ISSN: | 0969-9961, 1095-953X, 1095-953X |
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| Abstract | Mitochondrial defects are one of the common underlying causes of neuronal vulnerability in neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), and TDP-43 pathology is the most commonly observed proteinopathy. Disrupted inner mitochondrial membrane (IMM) reported in the upper motor neurons (UMNs) of ALS patients with TDP-43 pathology is recapitulated in the UMNs of well-characterized hTDP-43 mouse model of ALS. The construct validity, such as shared and common cellular pathology in mice and human, offers a unique opportunity to test treatment strategies that may translate to patients. SBT-272 is a well-tolerated brain-penetrant small molecule that stabilizes cardiolipin, a phospholipid found in IMM, thereby restoring mitochondrial structure and respiratory function. We investigated whether SBT-272 can improve IMM structure and health in UMNs diseased with TDP-43 pathology in our well-characterized UMN reporter line for ALS. We found that SBT-272 significantly improved mitochondrial structural integrity and restored mitochondrial motility and function. This led to improved health of diseased UMNs in vitro. In comparison to edaravone and AMX0035, SBT-272 appeared more effective in restoring health of diseased UMNs. Chronic treatment of SBT-272 for sixty days starting at an early symptomatic stage of the disease in vivo led to a significant reduction in astrogliosis, microgliosis, and TDP-43 pathology in the ALS motor cortex. Our results underscore the therapeutic potential of SBT-272, especially within the context of TDP-43 pathology and mitochondrial dysfunction.
•Early and progressive upper motor neuron (UMN) degeneration defines ALS pathology•Mitochondrial defects are prominent and common in UMNs with TDP-43 pathology•SBT-272 treatment improves mitochondrial stability, mobility and function•SBT-272 treatment reduces astrogliosis, microgliosis and TDP-43 pathology |
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| AbstractList | Mitochondrial defects are one of the common underlying causes of neuronal vulnerability in neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), and TDP-43 pathology is the most commonly observed proteinopathy. Disrupted inner mitochondrial membrane (IMM) reported in the upper motor neurons (UMNs) of ALS patients with TDP-43 pathology is recapitulated in the UMNs of well-characterized hTDP-43 mouse model of ALS. The construct validity, such as shared and common cellular pathology in mice and human, offers a unique opportunity to test treatment strategies that may translate to patients. SBT-272 is a well-tolerated brain-penetrant small molecule that stabilizes cardiolipin, a phospholipid found in IMM, thereby restoring mitochondrial structure and respiratory function. We investigated whether SBT-272 can improve IMM structure and health in UMNs diseased with TDP-43 pathology in our well-characterized UMN reporter line for ALS. We found that SBT-272 significantly improved mitochondrial structural integrity and restored mitochondrial motility and function. This led to improved health of diseased UMNs in vitro. In comparison to edaravone and AMX0035, SBT-272 appeared more effective in restoring health of diseased UMNs. Chronic treatment of SBT-272 for sixty days starting at an early symptomatic stage of the disease in vivo led to a significant reduction in astrogliosis, microgliosis, and TDP-43 pathology in the ALS motor cortex. Our results underscore the therapeutic potential of SBT-272, especially within the context of TDP-43 pathology and mitochondrial dysfunction. Mitochondrial defects are one of the common underlying causes of neuronal vulnerability in neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), and TDP-43 pathology is the most commonly observed proteinopathy. Disrupted inner mitochondrial membrane (IMM) reported in the upper motor neurons (UMNs) of ALS patients with TDP-43 pathology is recapitulated in the UMNs of well-characterized hTDP-43 mouse model of ALS. The construct validity, such as shared and common cellular pathology in mice and human, offers a unique opportunity to test treatment strategies that may translate to patients. SBT-272 is a well-tolerated brain-penetrant small molecule that stabilizes cardiolipin, a phospholipid found in IMM, thereby restoring mitochondrial structure and respiratory function. We investigated whether SBT-272 can improve IMM structure and health in UMNs diseased with TDP-43 pathology in our well-characterized UMN reporter line for ALS. We found that SBT-272 significantly improved mitochondrial structural integrity and restored mitochondrial motility and function. This led to improved health of diseased UMNs in vitro. In comparison to edaravone and AMX0035, SBT-272 appeared more effective in restoring health of diseased UMNs. Chronic treatment of SBT-272 for sixty days starting at an early symptomatic stage of the disease in vivo led to a significant reduction in astrogliosis, microgliosis, and TDP-43 pathology in the ALS motor cortex. Our results underscore the therapeutic potential of SBT-272, especially within the context of TDP-43 pathology and mitochondrial dysfunction.Mitochondrial defects are one of the common underlying causes of neuronal vulnerability in neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), and TDP-43 pathology is the most commonly observed proteinopathy. Disrupted inner mitochondrial membrane (IMM) reported in the upper motor neurons (UMNs) of ALS patients with TDP-43 pathology is recapitulated in the UMNs of well-characterized hTDP-43 mouse model of ALS. The construct validity, such as shared and common cellular pathology in mice and human, offers a unique opportunity to test treatment strategies that may translate to patients. SBT-272 is a well-tolerated brain-penetrant small molecule that stabilizes cardiolipin, a phospholipid found in IMM, thereby restoring mitochondrial structure and respiratory function. We investigated whether SBT-272 can improve IMM structure and health in UMNs diseased with TDP-43 pathology in our well-characterized UMN reporter line for ALS. We found that SBT-272 significantly improved mitochondrial structural integrity and restored mitochondrial motility and function. This led to improved health of diseased UMNs in vitro. In comparison to edaravone and AMX0035, SBT-272 appeared more effective in restoring health of diseased UMNs. Chronic treatment of SBT-272 for sixty days starting at an early symptomatic stage of the disease in vivo led to a significant reduction in astrogliosis, microgliosis, and TDP-43 pathology in the ALS motor cortex. Our results underscore the therapeutic potential of SBT-272, especially within the context of TDP-43 pathology and mitochondrial dysfunction. Mitochondrial defects are one of the common underlying causes of neuronal vulnerability in neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), and TDP-43 pathology is the most commonly observed proteinopathy. Disrupted inner mitochondrial membrane (IMM) reported in the upper motor neurons (UMNs) of ALS patients with TDP-43 pathology is recapitulated in the UMNs of well-characterized hTDP-43 mouse model of ALS. The construct validity, such as shared and common cellular pathology in mice and human, offers a unique opportunity to test treatment strategies that may translate to patients. SBT-272 is a well-tolerated brain-penetrant small molecule that stabilizes cardiolipin, a phospholipid found in IMM, thereby restoring mitochondrial structure and respiratory function. We investigated whether SBT-272 can improve IMM structure and health in UMNs diseased with TDP-43 pathology in our well-characterized UMN reporter line for ALS. We found that SBT-272 significantly improved mitochondrial structural integrity and restored mitochondrial motility and function. This led to improved health of diseased UMNs in vitro. In comparison to edaravone and AMX0035, SBT-272 appeared more effective in restoring health of diseased UMNs. Chronic treatment of SBT-272 for sixty days starting at an early symptomatic stage of the disease in vivo led to a significant reduction in astrogliosis, microgliosis, and TDP-43 pathology in the ALS motor cortex. Our results underscore the therapeutic potential of SBT-272, especially within the context of TDP-43 pathology and mitochondrial dysfunction. •Early and progressive upper motor neuron (UMN) degeneration defines ALS pathology•Mitochondrial defects are prominent and common in UMNs with TDP-43 pathology•SBT-272 treatment improves mitochondrial stability, mobility and function•SBT-272 treatment reduces astrogliosis, microgliosis and TDP-43 pathology |
| ArticleNumber | 106022 |
| Author | Ahrens, Angela Koçak, Nuran Gautam, Mukesh Makrecka-Kuka, Marina Zariwala, Hatim A. Kuka, Janis Redmon, Martin Günay, Aksu Genç, Barış Swaminathan, Suchitra Özdinler, P. Hande Keefe, Dennis Aguilar-Wickings, Izaak R. Helmold, Benjamin Zheng, Guozhu |
| Author_xml | – sequence: 1 givenname: Mukesh surname: Gautam fullname: Gautam, Mukesh organization: Department of Neurology, Feinberg School of Medicine, Northwestern University, 303 E Chicago Ave, Chicago, IL 60611, USA – sequence: 2 givenname: Barış surname: Genç fullname: Genç, Barış organization: Department of Neurology, Feinberg School of Medicine, Northwestern University, 303 E Chicago Ave, Chicago, IL 60611, USA – sequence: 3 givenname: Benjamin surname: Helmold fullname: Helmold, Benjamin organization: Department of Neurology, Feinberg School of Medicine, Northwestern University, 303 E Chicago Ave, Chicago, IL 60611, USA – sequence: 4 givenname: Angela surname: Ahrens fullname: Ahrens, Angela organization: Department of Neurology, Feinberg School of Medicine, Northwestern University, 303 E Chicago Ave, Chicago, IL 60611, USA – sequence: 5 givenname: Janis surname: Kuka fullname: Kuka, Janis organization: Latvian Institute of Organic Synthesis (LIOS), Aizkraukles Street 21, LV-2006 Riga, Latvia – sequence: 6 givenname: Marina surname: Makrecka-Kuka fullname: Makrecka-Kuka, Marina organization: Latvian Institute of Organic Synthesis (LIOS), Aizkraukles Street 21, LV-2006 Riga, Latvia – sequence: 7 givenname: Aksu surname: Günay fullname: Günay, Aksu organization: Department of Neurology, Feinberg School of Medicine, Northwestern University, 303 E Chicago Ave, Chicago, IL 60611, USA – sequence: 8 givenname: Nuran surname: Koçak fullname: Koçak, Nuran organization: Department of Neurology, Feinberg School of Medicine, Northwestern University, 303 E Chicago Ave, Chicago, IL 60611, USA – sequence: 9 givenname: Izaak R. surname: Aguilar-Wickings fullname: Aguilar-Wickings, Izaak R. organization: Department of Neurology, Feinberg School of Medicine, Northwestern University, 303 E Chicago Ave, Chicago, IL 60611, USA – sequence: 10 givenname: Dennis surname: Keefe fullname: Keefe, Dennis organization: Stealth BioTherapeutics, 140 Kendrick St Building C, Needham, MA 02494, USA – sequence: 11 givenname: Guozhu surname: Zheng fullname: Zheng, Guozhu organization: Stealth BioTherapeutics, 140 Kendrick St Building C, Needham, MA 02494, USA – sequence: 12 givenname: Suchitra surname: Swaminathan fullname: Swaminathan, Suchitra organization: Division of Rheumatology, Department of Medicine, Feinberg School of Medicine, Northwestern University, 420 E Superior St, Chicago, IL 60611, USA – sequence: 13 givenname: Martin surname: Redmon fullname: Redmon, Martin organization: Stealth BioTherapeutics, 140 Kendrick St Building C, Needham, MA 02494, USA – sequence: 14 givenname: Hatim A. surname: Zariwala fullname: Zariwala, Hatim A. organization: Stealth BioTherapeutics, 140 Kendrick St Building C, Needham, MA 02494, USA – sequence: 15 givenname: P. Hande surname: Özdinler fullname: Özdinler, P. Hande email: ozdinler@northwestern.edu organization: Department of Neurology, Feinberg School of Medicine, Northwestern University, 303 E Chicago Ave, Chicago, IL 60611, USA |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/36716828$$D View this record in MEDLINE/PubMed |
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| Cites_doi | 10.1007/s40263-022-00945-x 10.1007/s00415-011-6104-z 10.1002/mus.20489 10.1111/bph.12461 10.1055/s-2001-15260 10.1016/j.heares.2016.07.019 10.1016/j.jneumeth.2006.05.030 10.3390/brainsci11050619 10.1038/nm.4130 10.1111/j.1750-3639.2000.tb00278.x 10.1038/clpt.2014.174 10.1074/jbc.RA119.012094 10.1371/journal.pone.0140316 10.1002/ctm2.336 10.1007/978-1-4939-3816-2_37 10.1016/j.neuron.2014.01.034 10.1038/s41598-022-09332-4 10.1126/science.1154584 10.1126/science.1134108 10.3390/brainsci11050549 10.1016/j.jchemneu.2016.03.003 10.1242/jcs.01130 10.1016/j.neuron.2005.08.030 10.1016/bs.adgen.2015.07.001 10.1038/nri.2017.21 10.1038/s41598-022-08068-5 10.1038/s41590-019-0353-x 10.2741/2727 10.1038/s41586-018-0846-z 10.1074/jbc.M500356200 10.1146/annurev.neuro.31.060407.125547 10.15252/embr.201949799 10.1093/emboj/21.3.221 10.1016/j.ejphar.2013.12.006 10.1016/j.tem.2021.01.006 10.1016/S1474-4422(17)30115-1 10.3390/ijms22157781 10.1242/jcs.038950 10.1177/1087057112441972 10.1097/WCO.0000000000000983 10.1038/s41434-021-00303-4 10.1016/j.mcn.2019.103396 10.1007/s40268-022-00391-6 10.2174/0929866525666181101105209 10.1042/bse0470115 10.1177/1074248411419502 10.1038/nrneurol.2016.140 10.1056/NEJMoa1916945 10.1371/journal.pone.0258614 10.1016/j.expneurol.2008.07.017 10.1523/JNEUROSCI.2614-08.2008 10.1146/annurev-immunol-101819-082015 10.1001/jamaneurol.2015.1872 10.1016/j.redox.2019.101255 10.3389/fnins.2020.00363 10.1038/s42003-020-1101-3 10.1016/S0006-3495(03)74754-7 10.1523/JNEUROSCI.18-09-03241.1998 10.1097/nen.0b013e31802c396b 10.1007/s00401-018-1934-8 10.1093/jnen/nlx025 10.1016/j.neulet.2018.04.053 10.1016/j.abb.2018.10.013 10.1016/j.neuron.2013.07.033 10.1111/j.1469-7793.1999.001aa.x 10.3389/fnins.2020.00573 10.1523/JNEUROSCI.2037-14.2014 10.1016/j.bbrc.2006.10.093 10.1038/nn1789 10.1016/j.tcb.2020.09.004 10.1371/journal.pgen.1007947 10.1002/mus.23533 10.3389/fncel.2019.00489 10.3390/biom5031319 10.1242/dmm.048912 10.1093/hmg/ddt319 10.1007/s00401-013-1115-8 10.1080/17482960600881870 10.1136/jnnp-2022-329024 10.1016/j.bbamem.2019.183064 10.1097/SHK.0b013e318173ea0b 10.3390/brainsci11030300 10.1093/brain/awl172 10.1073/pnas.0908767106 10.1523/JNEUROSCI.2787-12.2013 10.1186/s12915-015-0201-x 10.1016/j.yexcr.2015.01.004 10.1002/mus.27569 10.3390/brainsci11030369 10.1016/j.cell.2020.02.051 10.1016/j.drudis.2018.01.027 10.1186/s12974-017-0896-4 10.1002/ana.21147 10.1186/s12974-019-1589-y 10.1007/978-1-4939-8873-0_13 10.1146/annurev-genom-091416-035426 10.1007/s12031-010-9467-1 10.1523/JNEUROSCI.1630-10.2010 |
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| Keywords | Proteinopathy VCP IHC Motor neuron disease ALS OXPHOS PFA TMPD ADP CTIP2 CLEM CSMN Electron transport chain mSOD1 BSA UCHL1 CHCHD3 ANOVA SFM SBT-272 eGFP GI P3 FTLD EM IMM CL OMM DIV ET-1 TDP-43 ROS UMN ROX Cardiolipin ATP |
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| References | Mackenzie (bb0285) 2007; 61 Gunes (bb0210) 2020; 14 Xu (bb0505) 2010; 30 Wilson (bb0495) 2011; 4 Brooks (bb0050) 2022; 17 Clark (bb0090) 2016; 76 Szeto (bb0430) 2018; 25 Genc (bb0195) 2022; 12 Wang (bb0465) 2016; 22 Jovicic, Gitler (bb0245) 2014; 81 Ayala (bb0030) 2008; 121 Craven (bb0100) 2017; 18 Hervias (bb0220) 2006; 33 Duchen, Szabadkai (bb0135) 2010; 47 Brunet (bb0060) 2020; 14 Allen (bb0010) 2020; 3 Breda (bb0040) 2019; 26 Wegorzewska (bb0475) 2009; 106 Buratti (bb0065) 2015; 91 Whitlon (bb0490) 2017; 349 Cykowski (bb0105) 2017; 76 O’Reilly (bb0335) 2003; 85 Cotelli (bb0095) 2013; 47 Geevasinga (bb0180) 2015; 72 Marques (bb0305) 2021; 11 Al-Ali (bb0005) 2016; 1462 Falabella (bb0145) 2021; 32 Miller, Sheetz (bb0310) 2004; 117 Sreedharan (bb0415) 2008; 319 Davis (bb0120) 2018; 678 Genge (bb0205) 2022; 22 Charcot, Marie (bb0080) 1885; 10 Bright (bb0045) 2021; 22 Gautam (bb0170) 2019; 13 Genc (bb0200) 2022; 29 Jara (bb0240) 2019; 16 Dadon-Nachum (bb0110) 2011; 43 Weinberg (bb0480) 2019; 565 Arai (bb0025) 2006; 351 Genc (bb0190) 2021; 11 Makrecka (bb0290) 2014; 723 Thomsen (bb0445) 2014; 34 Camilleri (bb0075) 2020; 1862 Rossmann (bb0390) 2021; 14 Sholl (bb0405) 1953; 87 Jara (bb0235) 2017; 14 Duchen (bb0130) 1999; 516 Gao (bb0160) 2019; 100 Manfredi, Beal (bb0300) 2000; 10 Ito (bb0230) 2008; 213 Steinert (bb0420) 2021; 39 Wang (bb0460) 2013; 22 Writing, Edaravone (bb0500) 2017; 16 David (bb0115) 2005; 280 Kong, Xu (bb0250) 1998; 18 Buratti, Baralle (bb0070) 2008; 13 Paumard (bb0365) 2002; 21 Fink (bb0155) 2013; 126 Brown, Robberecht (bb0055) 2001; 21 Soejima-Kusunoki (bb0410) 2022 Pizzuto, Pelegrin (bb0370) 2020; 30 Neumann (bb0325) 2006; 314 Paganoni (bb0360) 2022; 66 Szeto, Birk (bb0435) 2014; 96 Paganoni (bb0355) 2022; 93 West, Shadel (bb0485) 2017; 17 Sasaki, Iwata (bb0400) 2007; 66 Lemon (bb0265) 2008; 31 Yoshino, Kimura (bb0520) 2006; 7 Gautam (bb0165) 2019; 137 Appel (bb0015) 2021; 34 Ling (bb0280) 2013; 79 Paganoni, Cudkowicz (bb0345) 2020; 383 Arai, Waguri (bb0020) 2019; 1880 Geevasinga (bb0185) 2016; 12 Chicco, Sparagna (bb0085) 2007; 292 Szeto (bb0425) 2014; 171 Russell (bb0395) 2020; 181 Pradhan, Bellingham (bb0375) 2021; 11 Wang (bb0470) 2019; 15 Eisen (bb0140) 2021; 11 Lemon (bb0270) 2021; 11 Nguyen (bb0330) 2012; 17 Vucic, Kiernan (bb0455) 2006; 129 Paganoni (bb0350) 2020; 383 Szeto, Liu (bb0440) 2018; 660 Mitchell (bb0315) 2020; 295 Yasvoina (bb0510) 2013; 33 Billingham, Chandel (bb0035) 2019; 20 Yu (bb0525) 2020; 183 Gautam (bb0175) 2022; 12 Makrecka-Kuka (bb0295) 2015; 5 Lin, Sheng (bb0275) 2015; 334 Kuka (bb0260) 2012; 17 Heo (bb0215) 2022; 36 Dervishi, Ozdinler (bb0125) 2018; 23 Kuhlbrandt (bb0255) 2015; 13 Ozdinler, Macklis (bb0340) 2006; 9 Riley, Tait (bb0380) 2020; 21 Ristanovic (bb0385) 2006; 158 Ikeda, Iwasaki (bb0225) 2015; 10 Molyneaux (bb0320) 2005; 47 Yoshida (bb0515) 2008; 30 Verburg, Hollenbeck (bb0450) 2008; 28 Filosto (bb0150) 2011; 258 Miller (10.1016/j.nbd.2023.106022_bb0310) 2004; 117 Gao (10.1016/j.nbd.2023.106022_bb0160) 2019; 100 Fink (10.1016/j.nbd.2023.106022_bb0155) 2013; 126 Camilleri (10.1016/j.nbd.2023.106022_bb0075) 2020; 1862 Gautam (10.1016/j.nbd.2023.106022_bb0175) 2022; 12 Molyneaux (10.1016/j.nbd.2023.106022_bb0320) 2005; 47 Riley (10.1016/j.nbd.2023.106022_bb0380) 2020; 21 Gautam (10.1016/j.nbd.2023.106022_bb0170) 2019; 13 Pizzuto (10.1016/j.nbd.2023.106022_bb0370) 2020; 30 Yasvoina (10.1016/j.nbd.2023.106022_bb0510) 2013; 33 Szeto (10.1016/j.nbd.2023.106022_bb0425) 2014; 171 Billingham (10.1016/j.nbd.2023.106022_bb0035) 2019; 20 Mitchell (10.1016/j.nbd.2023.106022_bb0315) 2020; 295 Xu (10.1016/j.nbd.2023.106022_bb0505) 2010; 30 Gautam (10.1016/j.nbd.2023.106022_bb0165) 2019; 137 Yoshida (10.1016/j.nbd.2023.106022_bb0515) 2008; 30 Steinert (10.1016/j.nbd.2023.106022_bb0420) 2021; 39 Arai (10.1016/j.nbd.2023.106022_bb0020) 2019; 1880 Brunet (10.1016/j.nbd.2023.106022_bb0060) 2020; 14 Ayala (10.1016/j.nbd.2023.106022_bb0030) 2008; 121 Chicco (10.1016/j.nbd.2023.106022_bb0085) 2007; 292 Hervias (10.1016/j.nbd.2023.106022_bb0220) 2006; 33 Ling (10.1016/j.nbd.2023.106022_bb0280) 2013; 79 Szeto (10.1016/j.nbd.2023.106022_bb0430) 2018; 25 Davis (10.1016/j.nbd.2023.106022_bb0120) 2018; 678 Geevasinga (10.1016/j.nbd.2023.106022_bb0185) 2016; 12 Verburg (10.1016/j.nbd.2023.106022_bb0450) 2008; 28 O’Reilly (10.1016/j.nbd.2023.106022_bb0335) 2003; 85 Jara (10.1016/j.nbd.2023.106022_bb0235) 2017; 14 Makrecka (10.1016/j.nbd.2023.106022_bb0290) 2014; 723 Paganoni (10.1016/j.nbd.2023.106022_bb0355) 2022; 93 David (10.1016/j.nbd.2023.106022_bb0115) 2005; 280 Ikeda (10.1016/j.nbd.2023.106022_bb0225) 2015; 10 Kuka (10.1016/j.nbd.2023.106022_bb0260) 2012; 17 Writing (10.1016/j.nbd.2023.106022_bb0500) 2017; 16 Marques (10.1016/j.nbd.2023.106022_bb0305) 2021; 11 Heo (10.1016/j.nbd.2023.106022_bb0215) 2022; 36 Ozdinler (10.1016/j.nbd.2023.106022_bb0340) 2006; 9 Charcot (10.1016/j.nbd.2023.106022_bb0080) 1885; 10 Paganoni (10.1016/j.nbd.2023.106022_bb0345) 2020; 383 Genge (10.1016/j.nbd.2023.106022_bb0205) 2022; 22 Genc (10.1016/j.nbd.2023.106022_bb0200) 2022; 29 Geevasinga (10.1016/j.nbd.2023.106022_bb0180) 2015; 72 Wang (10.1016/j.nbd.2023.106022_bb0465) 2016; 22 Sreedharan (10.1016/j.nbd.2023.106022_bb0415) 2008; 319 Breda (10.1016/j.nbd.2023.106022_bb0040) 2019; 26 Craven (10.1016/j.nbd.2023.106022_bb0100) 2017; 18 Sasaki (10.1016/j.nbd.2023.106022_bb0400) 2007; 66 Szeto (10.1016/j.nbd.2023.106022_bb0435) 2014; 96 Mackenzie (10.1016/j.nbd.2023.106022_bb0285) 2007; 61 Lemon (10.1016/j.nbd.2023.106022_bb0265) 2008; 31 Rossmann (10.1016/j.nbd.2023.106022_bb0390) 2021; 14 Whitlon (10.1016/j.nbd.2023.106022_bb0490) 2017; 349 Szeto (10.1016/j.nbd.2023.106022_bb0440) 2018; 660 Genc (10.1016/j.nbd.2023.106022_bb0190) 2021; 11 Lin (10.1016/j.nbd.2023.106022_bb0275) 2015; 334 Paumard (10.1016/j.nbd.2023.106022_bb0365) 2002; 21 Soejima-Kusunoki (10.1016/j.nbd.2023.106022_bb0410) 2022 Jara (10.1016/j.nbd.2023.106022_bb0240) 2019; 16 Arai (10.1016/j.nbd.2023.106022_bb0025) 2006; 351 Buratti (10.1016/j.nbd.2023.106022_bb0065) 2015; 91 Weinberg (10.1016/j.nbd.2023.106022_bb0480) 2019; 565 Wang (10.1016/j.nbd.2023.106022_bb0460) 2013; 22 Russell (10.1016/j.nbd.2023.106022_bb0395) 2020; 181 Brooks (10.1016/j.nbd.2023.106022_bb0050) 2022; 17 Genc (10.1016/j.nbd.2023.106022_bb0195) 2022; 12 Bright (10.1016/j.nbd.2023.106022_bb0045) 2021; 22 Allen (10.1016/j.nbd.2023.106022_bb0010) 2020; 3 Nguyen (10.1016/j.nbd.2023.106022_bb0330) 2012; 17 Kong (10.1016/j.nbd.2023.106022_bb0250) 1998; 18 Duchen (10.1016/j.nbd.2023.106022_bb0135) 2010; 47 Yoshino (10.1016/j.nbd.2023.106022_bb0520) 2006; 7 Ristanovic (10.1016/j.nbd.2023.106022_bb0385) 2006; 158 Thomsen (10.1016/j.nbd.2023.106022_bb0445) 2014; 34 Buratti (10.1016/j.nbd.2023.106022_bb0070) 2008; 13 Brown (10.1016/j.nbd.2023.106022_bb0055) 2001; 21 Cotelli (10.1016/j.nbd.2023.106022_bb0095) 2013; 47 Appel (10.1016/j.nbd.2023.106022_bb0015) 2021; 34 Gunes (10.1016/j.nbd.2023.106022_bb0210) 2020; 14 Makrecka-Kuka (10.1016/j.nbd.2023.106022_bb0295) 2015; 5 Dadon-Nachum (10.1016/j.nbd.2023.106022_bb0110) 2011; 43 Wang (10.1016/j.nbd.2023.106022_bb0470) 2019; 15 Lemon (10.1016/j.nbd.2023.106022_bb0270) 2021; 11 Falabella (10.1016/j.nbd.2023.106022_bb0145) 2021; 32 Kuhlbrandt (10.1016/j.nbd.2023.106022_bb0255) 2015; 13 Wilson (10.1016/j.nbd.2023.106022_bb0495) 2011; 4 Paganoni (10.1016/j.nbd.2023.106022_bb0360) 2022; 66 Ito (10.1016/j.nbd.2023.106022_bb0230) 2008; 213 Neumann (10.1016/j.nbd.2023.106022_bb0325) 2006; 314 Manfredi (10.1016/j.nbd.2023.106022_bb0300) 2000; 10 Jovicic (10.1016/j.nbd.2023.106022_bb0245) 2014; 81 Wegorzewska (10.1016/j.nbd.2023.106022_bb0475) 2009; 106 Yu (10.1016/j.nbd.2023.106022_bb0525) 2020; 183 Sholl (10.1016/j.nbd.2023.106022_bb0405) 1953; 87 Dervishi (10.1016/j.nbd.2023.106022_bb0125) 2018; 23 Paganoni (10.1016/j.nbd.2023.106022_bb0350) 2020; 383 West (10.1016/j.nbd.2023.106022_bb0485) 2017; 17 Eisen (10.1016/j.nbd.2023.106022_bb0140) 2021; 11 Duchen (10.1016/j.nbd.2023.106022_bb0130) 1999; 516 Cykowski (10.1016/j.nbd.2023.106022_bb0105) 2017; 76 Vucic (10.1016/j.nbd.2023.106022_bb0455) 2006; 129 Clark (10.1016/j.nbd.2023.106022_bb0090) 2016; 76 Filosto (10.1016/j.nbd.2023.106022_bb0150) 2011; 258 Al-Ali (10.1016/j.nbd.2023.106022_bb0005) 2016; 1462 Pradhan (10.1016/j.nbd.2023.106022_bb0375) 2021; 11 |
| References_xml | – volume: 660 start-page: 137 year: 2018 end-page: 148 ident: bb0440 article-title: Cardiolipin-targeted peptides rejuvenate mitochondrial function, remodel mitochondria, and promote tissue regeneration during aging publication-title: Arch. Biochem. Biophys. – volume: 12 start-page: 651 year: 2016 end-page: 661 ident: bb0185 article-title: Pathophysiological and diagnostic implications of cortical dysfunction in ALS publication-title: Nat. Rev. Neurol. – volume: 7 start-page: 241 year: 2006 end-page: 245 ident: bb0520 article-title: Investigation of the therapeutic effects of edaravone, a free radical scavenger, on amyotrophic lateral sclerosis (Phase II study) publication-title: Amyotroph. Lateral Scler. – volume: 4 start-page: 147 year: 2011 end-page: 155 ident: bb0495 article-title: TDP-43 in aging and Alzheimer’s disease - a review publication-title: Int. J. Clin. Exp. Pathol. – volume: 11 year: 2021 ident: bb0190 article-title: Improving mitochondria and ER stability helps eliminate upper motor neuron degeneration that occurs due to mSOD1 toxicity and TDP-43 pathology publication-title: Clin Transl Med. – volume: 17 year: 2022 ident: bb0050 article-title: Edaravone efficacy in amyotrophic lateral sclerosis with reduced forced vital capacity: post-hoc analysis of study 19 (MCI186-19) [clinical trial NCT01492686] publication-title: PLoS One – volume: 33 start-page: 598 year: 2006 end-page: 608 ident: bb0220 article-title: Mitochondrial dysfunction and amyotrophic lateral sclerosis publication-title: Muscle Nerve – volume: 1862 year: 2020 ident: bb0075 article-title: Tau-induced mitochondrial membrane perturbation is dependent upon cardiolipin publication-title: Biochim. Biophys. Acta Biomembr. – volume: 319 start-page: 1668 year: 2008 end-page: 1672 ident: bb0415 article-title: TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis publication-title: Science. – volume: 85 start-page: 3350 year: 2003 end-page: 3357 ident: bb0335 article-title: Quantitative analysis of spontaneous mitochondrial depolarizations publication-title: Biophys. J. – start-page: 15 year: 2022 ident: bb0410 article-title: The protective effect of Edaravone on TDP-43 plus oxidative stress-induced neurotoxicity in neuronal cells: analysis of its neuroprotective mechanisms using RNA sequencing publication-title: Pharmaceuticals (Basel) – volume: 93 start-page: 871 year: 2022 end-page: 875 ident: bb0355 article-title: Effect of sodium phenylbutyrate/taurursodiol on tracheostomy/ventilation-free survival and hospitalisation in amyotrophic lateral sclerosis: long-term results from the CENTAUR trial publication-title: J. Neurol. Neurosurg. Psychiatry – volume: 21 start-page: 221 year: 2002 end-page: 230 ident: bb0365 article-title: The ATP synthase is involved in generating mitochondrial cristae morphology publication-title: EMBO J. – volume: 23 start-page: 696 year: 2018 end-page: 703 ident: bb0125 article-title: Incorporating upper motor neuron health in ALS drug discovery publication-title: Drug Discov. Today – volume: 314 start-page: 130 year: 2006 end-page: 133 ident: bb0325 article-title: Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis publication-title: Science. – volume: 66 start-page: 10 year: 2007 end-page: 16 ident: bb0400 article-title: Mitochondrial alterations in the spinal cord of patients with sporadic amyotrophic lateral sclerosis publication-title: J. Neuropathol. Exp. Neurol. – volume: 22 year: 2021 ident: bb0045 article-title: TDP-43 and inflammation: implications for amyotrophic lateral sclerosis and frontotemporal dementia publication-title: Int. J. Mol. Sci. – volume: 21 year: 2020 ident: bb0380 article-title: Mitochondrial DNA in inflammation and immunity publication-title: EMBO Rep. – volume: 18 start-page: 257 year: 2017 end-page: 275 ident: bb0100 article-title: Recent advances in mitochondrial disease publication-title: Annu. Rev. Genomics Hum. Genet. – volume: 29 start-page: 178 year: 2022 end-page: 192 ident: bb0200 article-title: Upper motor neurons are a target for gene therapy and UCHL1 is necessary and sufficient to improve cellular integrity of diseased upper motor neurons publication-title: Gene Ther. – volume: 349 start-page: 177 year: 2017 end-page: 181 ident: bb0490 article-title: Drug discovery for hearing loss: phenotypic screening of chemical compounds on primary cultures of the spiral ganglion publication-title: Hear. Res. – volume: 678 start-page: 8 year: 2018 end-page: 15 ident: bb0120 article-title: TDP-43 interacts with mitochondrial proteins critical for mitophagy and mitochondrial dynamics publication-title: Neurosci. Lett. – volume: 181 start-page: 168 year: 2020 end-page: 188 ident: bb0395 article-title: Mitochondrial diseases: Hope for the future publication-title: Cell. – volume: 30 start-page: 892 year: 2020 end-page: 903 ident: bb0370 article-title: Cardiolipin in immune signaling and cell death publication-title: Trends Cell Biol. – volume: 137 start-page: 47 year: 2019 end-page: 69 ident: bb0165 article-title: Mitochondria, ER, and nuclear membrane defects reveal early mechanisms for upper motor neuron vulnerability with respect to TDP-43 pathology publication-title: Acta Neuropathol. – volume: 32 start-page: 224 year: 2021 end-page: 237 ident: bb0145 article-title: Cardiolipin, mitochondria, and neurological disease publication-title: Trends Endocrinol. Metab. – volume: 11 year: 2021 ident: bb0305 article-title: Upper and lower motor neuron degenerations are Somatotopically related and temporally ordered in the Sod1 mouse model of amyotrophic lateral sclerosis publication-title: Brain Sci. – volume: 31 start-page: 195 year: 2008 end-page: 218 ident: bb0265 article-title: Descending pathways in motor control publication-title: Annu. Rev. Neurosci. – volume: 158 start-page: 212 year: 2006 end-page: 218 ident: bb0385 article-title: Application of modified Sholl analysis to neuronal dendritic arborization of the cat spinal cord publication-title: J. Neurosci. Methods – volume: 22 start-page: 205 year: 2022 end-page: 211 ident: bb0205 article-title: Analysis of the US safety data for Edaravone (Radicava((R))) from the third year after launch publication-title: Drugs R D. – volume: 383 start-page: 2294 year: 2020 ident: bb0345 article-title: Sodium Phenylbutyrate-Taurursodiol for ALS publication-title: Reply. N Engl J Med. – volume: 121 start-page: 3778 year: 2008 end-page: 3785 ident: bb0030 article-title: Structural determinants of the cellular localization and shuttling of TDP-43 publication-title: J. Cell Sci. – volume: 11 year: 2021 ident: bb0140 article-title: The dying forward hypothesis of ALS: tracing its history publication-title: Brain Sci. – volume: 34 start-page: 765 year: 2021 end-page: 772 ident: bb0015 article-title: Amyotrophic lateral sclerosis is a systemic disease: peripheral contributions to inflammation-mediated neurodegeneration publication-title: Curr. Opin. Neurol. – volume: 72 start-page: 1268 year: 2015 end-page: 1274 ident: bb0180 article-title: Cortical function in asymptomatic carriers and patients with C9orf72 amyotrophic lateral sclerosis publication-title: JAMA Neurol. – volume: 723 start-page: 55 year: 2014 end-page: 61 ident: bb0290 article-title: Mildronate, the inhibitor of L-carnitine transport, induces brain mitochondrial uncoupling and protects against anoxia-reoxygenation publication-title: Eur. J. Pharmacol. – volume: 565 start-page: 495 year: 2019 end-page: 499 ident: bb0480 article-title: Mitochondrial complex III is essential for suppressive function of regulatory T cells publication-title: Nature. – volume: 117 start-page: 2791 year: 2004 end-page: 2804 ident: bb0310 article-title: Axonal mitochondrial transport and potential are correlated publication-title: J. Cell Sci. – volume: 87 start-page: 387 year: 1953 end-page: 406 ident: bb0405 article-title: Dendritic organization in the neurons of the visual and motor cortices of the cat publication-title: J. Anat. – volume: 17 start-page: 215 year: 2012 end-page: 222 ident: bb0260 article-title: The cardioprotective effect of mildronate is diminished after co-treatment with L-carnitine publication-title: J. Cardiovasc. Pharmacol. Ther. – volume: 183 year: 2020 ident: bb0525 article-title: TDP-43 triggers mitochondrial DNA release via mPTP to activate cGAS/STING in ALS publication-title: Cell. – volume: 20 start-page: 380 year: 2019 end-page: 382 ident: bb0035 article-title: NAD-biosynthetic pathways regulate innate immunity publication-title: Nat. Immunol. – volume: 39 start-page: 395 year: 2021 end-page: 416 ident: bb0420 article-title: Mitochondrial metabolism regulation of T cell-mediated immunity publication-title: Annu. Rev. Immunol. – volume: 334 start-page: 35 year: 2015 end-page: 44 ident: bb0275 article-title: Regulation of mitochondrial transport in neurons publication-title: Exp. Cell Res. – volume: 171 start-page: 2029 year: 2014 end-page: 2050 ident: bb0425 article-title: First-in-class cardiolipin-protective compound as a therapeutic agent to restore mitochondrial bioenergetics publication-title: Br. J. Pharmacol. – volume: 33 start-page: 7890 year: 2013 end-page: 7904 ident: bb0510 article-title: eGFP expression under UCHL1 promoter genetically labels corticospinal motor neurons and a subpopulation of degeneration-resistant spinal motor neurons in an ALS mouse model publication-title: J. Neurosci. – volume: 100 year: 2019 ident: bb0160 article-title: TDP-43 proteinopathy and mitochondrial abnormalities in neurodegeneration publication-title: Mol. Cell. Neurosci. – volume: 47 start-page: 142 year: 2013 end-page: 143 ident: bb0095 article-title: Hemangioma of the semimembranosus muscle in a patient with late-onset glycogenosis II publication-title: Muscle Nerve – volume: 36 start-page: 1007 year: 2022 end-page: 1013 ident: bb0215 article-title: Sodium Phenylbutyrate and Ursodoxicoltaurine: first approval publication-title: CNS Drugs. – volume: 126 start-page: 307 year: 2013 end-page: 328 ident: bb0155 article-title: Hereditary spastic paraplegia: clinico-pathologic features and emerging molecular mechanisms publication-title: Acta Neuropathol. – volume: 17 start-page: 835 year: 2012 end-page: 842 ident: bb0330 article-title: Quantifying amyloid beta (Abeta)-mediated changes in neuronal morphology in primary cultures: implications for phenotypic screening publication-title: J. Biomol. Screen. – volume: 15 year: 2019 ident: bb0470 article-title: TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response publication-title: PLoS Genet. – volume: 14 start-page: 363 year: 2020 ident: bb0060 article-title: Cortical circuit dysfunction as a potential driver of amyotrophic lateral sclerosis publication-title: Front. Neurosci. – volume: 18 start-page: 3241 year: 1998 end-page: 3250 ident: bb0250 article-title: Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1 publication-title: J. Neurosci. – volume: 66 start-page: 136 year: 2022 end-page: 141 ident: bb0360 article-title: Survival analyses from the CENTAUR trial in amyotrophic lateral sclerosis: evaluating the impact of treatment crossover on outcomes publication-title: Muscle Nerve – volume: 383 start-page: 919 year: 2020 end-page: 930 ident: bb0350 article-title: Trial of sodium Phenylbutyrate-Taurursodiol for amyotrophic lateral sclerosis publication-title: N. Engl. J. Med. – volume: 30 start-page: 10851 year: 2010 end-page: 10859 ident: bb0505 article-title: Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice publication-title: J. Neurosci. – volume: 16 start-page: 505 year: 2017 end-page: 512 ident: bb0500 article-title: Safety and efficacy of edaravone in well defined patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled trial publication-title: Lancet Neurol. – volume: 30 start-page: 734 year: 2008 end-page: 739 ident: bb0515 article-title: Edaravone inhibits the induction of iNOS gene expression at transcriptional and posttranscriptional steps in murine macrophages publication-title: Shock. – volume: 10 start-page: 1 year: 1885 end-page: 35 ident: bb0080 article-title: Deux nouveaux cas de sclerose latérale amyotrophique suivis d’autopsie publication-title: Archives De Neurologie. – volume: 10 start-page: 462 year: 2000 end-page: 472 ident: bb0300 article-title: The role of mitochondria in the pathogenesis of neurodegenerative diseases publication-title: Brain Pathol. – volume: 21 start-page: 131 year: 2001 end-page: 139 ident: bb0055 article-title: Amyotrophic lateral sclerosis: pathogenesis publication-title: Semin. Neurol. – volume: 5 start-page: 1319 year: 2015 end-page: 1338 ident: bb0295 article-title: High-resolution Respirometry for simultaneous measurement of oxygen and hydrogen peroxide fluxes in Permeabilized cells, tissue homogenate and isolated mitochondria publication-title: Biomolecules. – volume: 292 start-page: C33 year: 2007 end-page: C44 ident: bb0085 article-title: Role of cardiolipin alterations in mitochondrial dysfunction and disease publication-title: Am. J. Phys. Cell Phys. – volume: 25 start-page: 1108 year: 2018 end-page: 1123 ident: bb0430 article-title: Stealth peptides target cellular powerhouses to fight rare and common age-related diseases publication-title: Protein Pept. Lett. – volume: 258 start-page: 1763 year: 2011 end-page: 1774 ident: bb0150 article-title: The role of mitochondria in neurodegenerative diseases publication-title: J. Neurol. – volume: 12 start-page: 4287 year: 2022 ident: bb0175 article-title: Mitochondrial dysregulation occurs early in ALS motor cortex with TDP-43 pathology and suggests maintaining NAD(+) balance as a therapeutic strategy publication-title: Sci. Rep. – volume: 13 start-page: 867 year: 2008 end-page: 878 ident: bb0070 article-title: Multiple roles of TDP-43 in gene expression, splicing regulation, and human disease publication-title: Front. Biosci. – volume: 81 start-page: 463 year: 2014 end-page: 465 ident: bb0245 article-title: TDP-43 in ALS: stay on target...Almost there publication-title: Neuron. – volume: 96 start-page: 672 year: 2014 end-page: 683 ident: bb0435 article-title: Serendipity and the discovery of novel compounds that restore mitochondrial plasticity publication-title: Clin. Pharmacol. Ther. – volume: 295 start-page: 7452 year: 2020 end-page: 7469 ident: bb0315 article-title: The mitochondria-targeted peptide SS-31 binds lipid bilayers and modulates surface electrostatics as a key component of its mechanism of action publication-title: J. Biol. Chem. – volume: 17 start-page: 363 year: 2017 end-page: 375 ident: bb0485 article-title: Mitochondrial DNA in innate immune responses and inflammatory pathology publication-title: Nat. Rev. Immunol. – volume: 16 start-page: 196 year: 2019 ident: bb0240 article-title: MCP1-CCR2 and neuroinflammation in the ALS motor cortex with TDP-43 pathology publication-title: J. Neuroinflammation – volume: 43 start-page: 470 year: 2011 end-page: 477 ident: bb0110 article-title: The “dying-back” phenomenon of motor neurons in ALS publication-title: J. Mol. Neurosci. – volume: 22 start-page: 4706 year: 2013 end-page: 4719 ident: bb0460 article-title: The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons publication-title: Hum. Mol. Genet. – volume: 10 year: 2015 ident: bb0225 article-title: Edaravone, a free radical scavenger, delayed symptomatic and pathological progression of motor neuron disease in the wobbler mouse publication-title: PLoS One – volume: 14 start-page: 573 year: 2020 ident: bb0210 article-title: Exciting complexity: the role of motor circuit elements in ALS pathophysiology publication-title: Front. Neurosci. – volume: 1880 start-page: 211 year: 2019 end-page: 221 ident: bb0020 article-title: Improved Electron microscopy fixation methods for tracking autophagy-associated membranes in cultured mammalian cells publication-title: Methods Mol. Biol. – volume: 61 start-page: 427 year: 2007 end-page: 434 ident: bb0285 article-title: Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations publication-title: Ann. Neurol. – volume: 351 start-page: 602 year: 2006 end-page: 611 ident: bb0025 article-title: TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis publication-title: Biochem. Biophys. Res. Commun. – volume: 516 start-page: 1 year: 1999 end-page: 17 ident: bb0130 article-title: Contributions of mitochondria to animal physiology: from homeostatic sensor to calcium signalling and cell death publication-title: J. Physiol. – volume: 13 start-page: 489 year: 2019 ident: bb0170 article-title: Mitoautophagy: a unique self-destructive path mitochondria of upper motor neurons with TDP-43 pathology take, very early in ALS publication-title: Front. Cell. Neurosci. – volume: 34 start-page: 15587 year: 2014 end-page: 15600 ident: bb0445 article-title: Delayed disease onset and extended survival in the SOD1G93A rat model of amyotrophic lateral sclerosis after suppression of mutant SOD1 in the motor cortex publication-title: J. Neurosci. – volume: 47 start-page: 115 year: 2010 end-page: 137 ident: bb0135 article-title: Roles of mitochondria in human disease publication-title: Essays Biochem. – volume: 47 start-page: 817 year: 2005 end-page: 831 ident: bb0320 article-title: Fezl is required for the birth and specification of corticospinal motor neurons publication-title: Neuron. – volume: 11 year: 2021 ident: bb0375 article-title: Neurophysiological mechanisms underlying cortical hyper-excitability in amyotrophic lateral sclerosis: a review publication-title: Brain Sci. – volume: 11 year: 2021 ident: bb0270 article-title: The cortical “upper Motoneuron” in health and disease publication-title: Brain Sci. – volume: 76 start-page: 402 year: 2017 end-page: 413 ident: bb0105 article-title: Clinical significance of TDP-43 neuropathology in amyotrophic lateral sclerosis publication-title: J. Neuropathol. Exp. Neurol. – volume: 129 start-page: 2436 year: 2006 end-page: 2446 ident: bb0455 article-title: Novel threshold tracking techniques suggest that cortical hyperexcitability is an early feature of motor neuron disease publication-title: Brain. – volume: 213 start-page: 448 year: 2008 end-page: 455 ident: bb0230 article-title: Treatment with edaravone, initiated at symptom onset, slows motor decline and decreases SOD1 deposition in ALS mice publication-title: Exp. Neurol. – volume: 1462 start-page: 677 year: 2016 end-page: 688 ident: bb0005 article-title: Phenotypic screening of small-molecule inhibitors: implications for therapeutic discovery and drug target development in traumatic brain injury publication-title: Methods Mol. Biol. – volume: 280 start-page: 23802 year: 2005 end-page: 23814 ident: bb0115 article-title: Proteomic and functional analyses reveal a mitochondrial dysfunction in P301L tau transgenic mice publication-title: J. Biol. Chem. – volume: 91 start-page: 1 year: 2015 end-page: 53 ident: bb0065 article-title: Functional significance of TDP-43 mutations in disease publication-title: Adv. Genet. – volume: 12 start-page: 5383 year: 2022 ident: bb0195 article-title: NU-9 improves health of hSOD1(G93A) mouse upper motor neurons in vitro, especially in combination with riluzole or edaravone publication-title: Sci. Rep. – volume: 3 start-page: 389 year: 2020 ident: bb0010 article-title: The cardiolipin-binding peptide elamipretide mitigates fragmentation of cristae networks following cardiac ischemia reperfusion in rats publication-title: Commun Biol. – volume: 26 year: 2019 ident: bb0040 article-title: Mitochondria as central hub of the immune system publication-title: Redox Biol. – volume: 106 start-page: 18809 year: 2009 end-page: 18814 ident: bb0475 article-title: TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration publication-title: Proc. Natl. Acad. Sci. U. S. A. – volume: 14 start-page: 129 year: 2017 ident: bb0235 article-title: Evidence for an early innate immune response in the motor cortex of ALS publication-title: J. Neuroinflammation – volume: 28 start-page: 8306 year: 2008 end-page: 8315 ident: bb0450 article-title: Mitochondrial membrane potential in axons increases with local nerve growth factor or semaphorin signaling publication-title: J. Neurosci. – volume: 14 year: 2021 ident: bb0390 article-title: Mitochondrial function in development and disease publication-title: Dis. Model. Mech. – volume: 79 start-page: 416 year: 2013 end-page: 438 ident: bb0280 article-title: Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis publication-title: Neuron. – volume: 22 start-page: 869 year: 2016 end-page: 878 ident: bb0465 article-title: The inhibition of TDP-43 mitochondrial localization blocks its neuronal toxicity publication-title: Nat. Med. – volume: 9 start-page: 1371 year: 2006 end-page: 1381 ident: bb0340 article-title: IGF-I specifically enhances axon outgrowth of corticospinal motor neurons publication-title: Nat. Neurosci. – volume: 76 start-page: 35 year: 2016 end-page: 47 ident: bb0090 article-title: Axonal degeneration, distal collateral branching and neuromuscular junction architecture alterations occur prior to symptom onset in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis publication-title: J. Chem. Neuroanat. – volume: 13 start-page: 89 year: 2015 ident: bb0255 article-title: Structure and function of mitochondrial membrane protein complexes publication-title: BMC Biol. – volume: 36 start-page: 1007 year: 2022 ident: 10.1016/j.nbd.2023.106022_bb0215 article-title: Sodium Phenylbutyrate and Ursodoxicoltaurine: first approval publication-title: CNS Drugs. doi: 10.1007/s40263-022-00945-x – volume: 258 start-page: 1763 year: 2011 ident: 10.1016/j.nbd.2023.106022_bb0150 article-title: The role of mitochondria in neurodegenerative diseases publication-title: J. Neurol. doi: 10.1007/s00415-011-6104-z – volume: 33 start-page: 598 year: 2006 ident: 10.1016/j.nbd.2023.106022_bb0220 article-title: Mitochondrial dysfunction and amyotrophic lateral sclerosis publication-title: Muscle Nerve doi: 10.1002/mus.20489 – volume: 171 start-page: 2029 year: 2014 ident: 10.1016/j.nbd.2023.106022_bb0425 article-title: First-in-class cardiolipin-protective compound as a therapeutic agent to restore mitochondrial bioenergetics publication-title: Br. J. Pharmacol. doi: 10.1111/bph.12461 – volume: 21 start-page: 131 year: 2001 ident: 10.1016/j.nbd.2023.106022_bb0055 article-title: Amyotrophic lateral sclerosis: pathogenesis publication-title: Semin. Neurol. doi: 10.1055/s-2001-15260 – volume: 349 start-page: 177 year: 2017 ident: 10.1016/j.nbd.2023.106022_bb0490 article-title: Drug discovery for hearing loss: phenotypic screening of chemical compounds on primary cultures of the spiral ganglion publication-title: Hear. Res. doi: 10.1016/j.heares.2016.07.019 – volume: 158 start-page: 212 year: 2006 ident: 10.1016/j.nbd.2023.106022_bb0385 article-title: Application of modified Sholl analysis to neuronal dendritic arborization of the cat spinal cord publication-title: J. Neurosci. Methods doi: 10.1016/j.jneumeth.2006.05.030 – start-page: 15 year: 2022 ident: 10.1016/j.nbd.2023.106022_bb0410 article-title: The protective effect of Edaravone on TDP-43 plus oxidative stress-induced neurotoxicity in neuronal cells: analysis of its neuroprotective mechanisms using RNA sequencing publication-title: Pharmaceuticals (Basel) – volume: 87 start-page: 387 year: 1953 ident: 10.1016/j.nbd.2023.106022_bb0405 article-title: Dendritic organization in the neurons of the visual and motor cortices of the cat publication-title: J. Anat. – volume: 11 year: 2021 ident: 10.1016/j.nbd.2023.106022_bb0270 article-title: The cortical “upper Motoneuron” in health and disease publication-title: Brain Sci. doi: 10.3390/brainsci11050619 – volume: 22 start-page: 869 year: 2016 ident: 10.1016/j.nbd.2023.106022_bb0465 article-title: The inhibition of TDP-43 mitochondrial localization blocks its neuronal toxicity publication-title: Nat. Med. doi: 10.1038/nm.4130 – volume: 10 start-page: 462 year: 2000 ident: 10.1016/j.nbd.2023.106022_bb0300 article-title: The role of mitochondria in the pathogenesis of neurodegenerative diseases publication-title: Brain Pathol. doi: 10.1111/j.1750-3639.2000.tb00278.x – volume: 96 start-page: 672 year: 2014 ident: 10.1016/j.nbd.2023.106022_bb0435 article-title: Serendipity and the discovery of novel compounds that restore mitochondrial plasticity publication-title: Clin. Pharmacol. Ther. doi: 10.1038/clpt.2014.174 – volume: 295 start-page: 7452 year: 2020 ident: 10.1016/j.nbd.2023.106022_bb0315 article-title: The mitochondria-targeted peptide SS-31 binds lipid bilayers and modulates surface electrostatics as a key component of its mechanism of action publication-title: J. Biol. Chem. doi: 10.1074/jbc.RA119.012094 – volume: 10 year: 2015 ident: 10.1016/j.nbd.2023.106022_bb0225 article-title: Edaravone, a free radical scavenger, delayed symptomatic and pathological progression of motor neuron disease in the wobbler mouse publication-title: PLoS One doi: 10.1371/journal.pone.0140316 – volume: 11 year: 2021 ident: 10.1016/j.nbd.2023.106022_bb0190 article-title: Improving mitochondria and ER stability helps eliminate upper motor neuron degeneration that occurs due to mSOD1 toxicity and TDP-43 pathology publication-title: Clin Transl Med. doi: 10.1002/ctm2.336 – volume: 1462 start-page: 677 year: 2016 ident: 10.1016/j.nbd.2023.106022_bb0005 article-title: Phenotypic screening of small-molecule inhibitors: implications for therapeutic discovery and drug target development in traumatic brain injury publication-title: Methods Mol. Biol. doi: 10.1007/978-1-4939-3816-2_37 – volume: 81 start-page: 463 year: 2014 ident: 10.1016/j.nbd.2023.106022_bb0245 article-title: TDP-43 in ALS: stay on target...Almost there publication-title: Neuron. doi: 10.1016/j.neuron.2014.01.034 – volume: 12 start-page: 5383 year: 2022 ident: 10.1016/j.nbd.2023.106022_bb0195 article-title: NU-9 improves health of hSOD1(G93A) mouse upper motor neurons in vitro, especially in combination with riluzole or edaravone publication-title: Sci. Rep. doi: 10.1038/s41598-022-09332-4 – volume: 319 start-page: 1668 year: 2008 ident: 10.1016/j.nbd.2023.106022_bb0415 article-title: TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis publication-title: Science. doi: 10.1126/science.1154584 – volume: 314 start-page: 130 year: 2006 ident: 10.1016/j.nbd.2023.106022_bb0325 article-title: Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis publication-title: Science. doi: 10.1126/science.1134108 – volume: 11 year: 2021 ident: 10.1016/j.nbd.2023.106022_bb0375 article-title: Neurophysiological mechanisms underlying cortical hyper-excitability in amyotrophic lateral sclerosis: a review publication-title: Brain Sci. doi: 10.3390/brainsci11050549 – volume: 76 start-page: 35 year: 2016 ident: 10.1016/j.nbd.2023.106022_bb0090 article-title: Axonal degeneration, distal collateral branching and neuromuscular junction architecture alterations occur prior to symptom onset in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis publication-title: J. Chem. Neuroanat. doi: 10.1016/j.jchemneu.2016.03.003 – volume: 117 start-page: 2791 year: 2004 ident: 10.1016/j.nbd.2023.106022_bb0310 article-title: Axonal mitochondrial transport and potential are correlated publication-title: J. Cell Sci. doi: 10.1242/jcs.01130 – volume: 47 start-page: 817 year: 2005 ident: 10.1016/j.nbd.2023.106022_bb0320 article-title: Fezl is required for the birth and specification of corticospinal motor neurons publication-title: Neuron. doi: 10.1016/j.neuron.2005.08.030 – volume: 91 start-page: 1 year: 2015 ident: 10.1016/j.nbd.2023.106022_bb0065 article-title: Functional significance of TDP-43 mutations in disease publication-title: Adv. Genet. doi: 10.1016/bs.adgen.2015.07.001 – volume: 183 issue: 636–649 year: 2020 ident: 10.1016/j.nbd.2023.106022_bb0525 article-title: TDP-43 triggers mitochondrial DNA release via mPTP to activate cGAS/STING in ALS publication-title: Cell. – volume: 17 start-page: 363 year: 2017 ident: 10.1016/j.nbd.2023.106022_bb0485 article-title: Mitochondrial DNA in innate immune responses and inflammatory pathology publication-title: Nat. Rev. Immunol. doi: 10.1038/nri.2017.21 – volume: 12 start-page: 4287 year: 2022 ident: 10.1016/j.nbd.2023.106022_bb0175 article-title: Mitochondrial dysregulation occurs early in ALS motor cortex with TDP-43 pathology and suggests maintaining NAD(+) balance as a therapeutic strategy publication-title: Sci. Rep. doi: 10.1038/s41598-022-08068-5 – volume: 20 start-page: 380 year: 2019 ident: 10.1016/j.nbd.2023.106022_bb0035 article-title: NAD-biosynthetic pathways regulate innate immunity publication-title: Nat. Immunol. doi: 10.1038/s41590-019-0353-x – volume: 13 start-page: 867 year: 2008 ident: 10.1016/j.nbd.2023.106022_bb0070 article-title: Multiple roles of TDP-43 in gene expression, splicing regulation, and human disease publication-title: Front. Biosci. doi: 10.2741/2727 – volume: 565 start-page: 495 year: 2019 ident: 10.1016/j.nbd.2023.106022_bb0480 article-title: Mitochondrial complex III is essential for suppressive function of regulatory T cells publication-title: Nature. doi: 10.1038/s41586-018-0846-z – volume: 280 start-page: 23802 year: 2005 ident: 10.1016/j.nbd.2023.106022_bb0115 article-title: Proteomic and functional analyses reveal a mitochondrial dysfunction in P301L tau transgenic mice publication-title: J. Biol. Chem. doi: 10.1074/jbc.M500356200 – volume: 31 start-page: 195 year: 2008 ident: 10.1016/j.nbd.2023.106022_bb0265 article-title: Descending pathways in motor control publication-title: Annu. Rev. Neurosci. doi: 10.1146/annurev.neuro.31.060407.125547 – volume: 21 year: 2020 ident: 10.1016/j.nbd.2023.106022_bb0380 article-title: Mitochondrial DNA in inflammation and immunity publication-title: EMBO Rep. doi: 10.15252/embr.201949799 – volume: 21 start-page: 221 year: 2002 ident: 10.1016/j.nbd.2023.106022_bb0365 article-title: The ATP synthase is involved in generating mitochondrial cristae morphology publication-title: EMBO J. doi: 10.1093/emboj/21.3.221 – volume: 723 start-page: 55 year: 2014 ident: 10.1016/j.nbd.2023.106022_bb0290 article-title: Mildronate, the inhibitor of L-carnitine transport, induces brain mitochondrial uncoupling and protects against anoxia-reoxygenation publication-title: Eur. J. Pharmacol. doi: 10.1016/j.ejphar.2013.12.006 – volume: 32 start-page: 224 year: 2021 ident: 10.1016/j.nbd.2023.106022_bb0145 article-title: Cardiolipin, mitochondria, and neurological disease publication-title: Trends Endocrinol. Metab. doi: 10.1016/j.tem.2021.01.006 – volume: 16 start-page: 505 year: 2017 ident: 10.1016/j.nbd.2023.106022_bb0500 article-title: Safety and efficacy of edaravone in well defined patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled trial publication-title: Lancet Neurol. doi: 10.1016/S1474-4422(17)30115-1 – volume: 22 year: 2021 ident: 10.1016/j.nbd.2023.106022_bb0045 article-title: TDP-43 and inflammation: implications for amyotrophic lateral sclerosis and frontotemporal dementia publication-title: Int. J. Mol. Sci. doi: 10.3390/ijms22157781 – volume: 121 start-page: 3778 year: 2008 ident: 10.1016/j.nbd.2023.106022_bb0030 article-title: Structural determinants of the cellular localization and shuttling of TDP-43 publication-title: J. Cell Sci. doi: 10.1242/jcs.038950 – volume: 17 start-page: 835 year: 2012 ident: 10.1016/j.nbd.2023.106022_bb0330 article-title: Quantifying amyloid beta (Abeta)-mediated changes in neuronal morphology in primary cultures: implications for phenotypic screening publication-title: J. Biomol. Screen. doi: 10.1177/1087057112441972 – volume: 34 start-page: 765 year: 2021 ident: 10.1016/j.nbd.2023.106022_bb0015 article-title: Amyotrophic lateral sclerosis is a systemic disease: peripheral contributions to inflammation-mediated neurodegeneration publication-title: Curr. Opin. Neurol. doi: 10.1097/WCO.0000000000000983 – volume: 29 start-page: 178 year: 2022 ident: 10.1016/j.nbd.2023.106022_bb0200 article-title: Upper motor neurons are a target for gene therapy and UCHL1 is necessary and sufficient to improve cellular integrity of diseased upper motor neurons publication-title: Gene Ther. doi: 10.1038/s41434-021-00303-4 – volume: 100 year: 2019 ident: 10.1016/j.nbd.2023.106022_bb0160 article-title: TDP-43 proteinopathy and mitochondrial abnormalities in neurodegeneration publication-title: Mol. Cell. Neurosci. doi: 10.1016/j.mcn.2019.103396 – volume: 22 start-page: 205 issue: 3 year: 2022 ident: 10.1016/j.nbd.2023.106022_bb0205 article-title: Analysis of the US safety data for Edaravone (Radicava((R))) from the third year after launch publication-title: Drugs R D. doi: 10.1007/s40268-022-00391-6 – volume: 25 start-page: 1108 year: 2018 ident: 10.1016/j.nbd.2023.106022_bb0430 article-title: Stealth peptides target cellular powerhouses to fight rare and common age-related diseases publication-title: Protein Pept. Lett. doi: 10.2174/0929866525666181101105209 – volume: 47 start-page: 115 year: 2010 ident: 10.1016/j.nbd.2023.106022_bb0135 article-title: Roles of mitochondria in human disease publication-title: Essays Biochem. doi: 10.1042/bse0470115 – volume: 383 start-page: 2294 year: 2020 ident: 10.1016/j.nbd.2023.106022_bb0345 article-title: Sodium Phenylbutyrate-Taurursodiol for ALS publication-title: Reply. N Engl J Med. – volume: 17 start-page: 215 year: 2012 ident: 10.1016/j.nbd.2023.106022_bb0260 article-title: The cardioprotective effect of mildronate is diminished after co-treatment with L-carnitine publication-title: J. Cardiovasc. Pharmacol. Ther. doi: 10.1177/1074248411419502 – volume: 12 start-page: 651 year: 2016 ident: 10.1016/j.nbd.2023.106022_bb0185 article-title: Pathophysiological and diagnostic implications of cortical dysfunction in ALS publication-title: Nat. Rev. Neurol. doi: 10.1038/nrneurol.2016.140 – volume: 383 start-page: 919 year: 2020 ident: 10.1016/j.nbd.2023.106022_bb0350 article-title: Trial of sodium Phenylbutyrate-Taurursodiol for amyotrophic lateral sclerosis publication-title: N. Engl. J. Med. doi: 10.1056/NEJMoa1916945 – volume: 17 year: 2022 ident: 10.1016/j.nbd.2023.106022_bb0050 article-title: Edaravone efficacy in amyotrophic lateral sclerosis with reduced forced vital capacity: post-hoc analysis of study 19 (MCI186-19) [clinical trial NCT01492686] publication-title: PLoS One doi: 10.1371/journal.pone.0258614 – volume: 213 start-page: 448 year: 2008 ident: 10.1016/j.nbd.2023.106022_bb0230 article-title: Treatment with edaravone, initiated at symptom onset, slows motor decline and decreases SOD1 deposition in ALS mice publication-title: Exp. Neurol. doi: 10.1016/j.expneurol.2008.07.017 – volume: 28 start-page: 8306 year: 2008 ident: 10.1016/j.nbd.2023.106022_bb0450 article-title: Mitochondrial membrane potential in axons increases with local nerve growth factor or semaphorin signaling publication-title: J. Neurosci. doi: 10.1523/JNEUROSCI.2614-08.2008 – volume: 39 start-page: 395 year: 2021 ident: 10.1016/j.nbd.2023.106022_bb0420 article-title: Mitochondrial metabolism regulation of T cell-mediated immunity publication-title: Annu. Rev. Immunol. doi: 10.1146/annurev-immunol-101819-082015 – volume: 72 start-page: 1268 year: 2015 ident: 10.1016/j.nbd.2023.106022_bb0180 article-title: Cortical function in asymptomatic carriers and patients with C9orf72 amyotrophic lateral sclerosis publication-title: JAMA Neurol. doi: 10.1001/jamaneurol.2015.1872 – volume: 26 year: 2019 ident: 10.1016/j.nbd.2023.106022_bb0040 article-title: Mitochondria as central hub of the immune system publication-title: Redox Biol. doi: 10.1016/j.redox.2019.101255 – volume: 14 start-page: 363 year: 2020 ident: 10.1016/j.nbd.2023.106022_bb0060 article-title: Cortical circuit dysfunction as a potential driver of amyotrophic lateral sclerosis publication-title: Front. Neurosci. doi: 10.3389/fnins.2020.00363 – volume: 3 start-page: 389 year: 2020 ident: 10.1016/j.nbd.2023.106022_bb0010 article-title: The cardiolipin-binding peptide elamipretide mitigates fragmentation of cristae networks following cardiac ischemia reperfusion in rats publication-title: Commun Biol. doi: 10.1038/s42003-020-1101-3 – volume: 85 start-page: 3350 year: 2003 ident: 10.1016/j.nbd.2023.106022_bb0335 article-title: Quantitative analysis of spontaneous mitochondrial depolarizations publication-title: Biophys. J. doi: 10.1016/S0006-3495(03)74754-7 – volume: 18 start-page: 3241 year: 1998 ident: 10.1016/j.nbd.2023.106022_bb0250 article-title: Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1 publication-title: J. Neurosci. doi: 10.1523/JNEUROSCI.18-09-03241.1998 – volume: 66 start-page: 10 year: 2007 ident: 10.1016/j.nbd.2023.106022_bb0400 article-title: Mitochondrial alterations in the spinal cord of patients with sporadic amyotrophic lateral sclerosis publication-title: J. Neuropathol. Exp. Neurol. doi: 10.1097/nen.0b013e31802c396b – volume: 137 start-page: 47 year: 2019 ident: 10.1016/j.nbd.2023.106022_bb0165 article-title: Mitochondria, ER, and nuclear membrane defects reveal early mechanisms for upper motor neuron vulnerability with respect to TDP-43 pathology publication-title: Acta Neuropathol. doi: 10.1007/s00401-018-1934-8 – volume: 4 start-page: 147 year: 2011 ident: 10.1016/j.nbd.2023.106022_bb0495 article-title: TDP-43 in aging and Alzheimer’s disease - a review publication-title: Int. J. Clin. Exp. Pathol. – volume: 76 start-page: 402 year: 2017 ident: 10.1016/j.nbd.2023.106022_bb0105 article-title: Clinical significance of TDP-43 neuropathology in amyotrophic lateral sclerosis publication-title: J. Neuropathol. Exp. Neurol. doi: 10.1093/jnen/nlx025 – volume: 678 start-page: 8 year: 2018 ident: 10.1016/j.nbd.2023.106022_bb0120 article-title: TDP-43 interacts with mitochondrial proteins critical for mitophagy and mitochondrial dynamics publication-title: Neurosci. Lett. doi: 10.1016/j.neulet.2018.04.053 – volume: 660 start-page: 137 year: 2018 ident: 10.1016/j.nbd.2023.106022_bb0440 article-title: Cardiolipin-targeted peptides rejuvenate mitochondrial function, remodel mitochondria, and promote tissue regeneration during aging publication-title: Arch. Biochem. Biophys. doi: 10.1016/j.abb.2018.10.013 – volume: 79 start-page: 416 year: 2013 ident: 10.1016/j.nbd.2023.106022_bb0280 article-title: Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis publication-title: Neuron. doi: 10.1016/j.neuron.2013.07.033 – volume: 516 start-page: 1 issue: Pt 1 year: 1999 ident: 10.1016/j.nbd.2023.106022_bb0130 article-title: Contributions of mitochondria to animal physiology: from homeostatic sensor to calcium signalling and cell death publication-title: J. Physiol. doi: 10.1111/j.1469-7793.1999.001aa.x – volume: 14 start-page: 573 year: 2020 ident: 10.1016/j.nbd.2023.106022_bb0210 article-title: Exciting complexity: the role of motor circuit elements in ALS pathophysiology publication-title: Front. Neurosci. doi: 10.3389/fnins.2020.00573 – volume: 34 start-page: 15587 year: 2014 ident: 10.1016/j.nbd.2023.106022_bb0445 article-title: Delayed disease onset and extended survival in the SOD1G93A rat model of amyotrophic lateral sclerosis after suppression of mutant SOD1 in the motor cortex publication-title: J. Neurosci. doi: 10.1523/JNEUROSCI.2037-14.2014 – volume: 351 start-page: 602 year: 2006 ident: 10.1016/j.nbd.2023.106022_bb0025 article-title: TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis publication-title: Biochem. Biophys. Res. Commun. doi: 10.1016/j.bbrc.2006.10.093 – volume: 9 start-page: 1371 year: 2006 ident: 10.1016/j.nbd.2023.106022_bb0340 article-title: IGF-I specifically enhances axon outgrowth of corticospinal motor neurons publication-title: Nat. Neurosci. doi: 10.1038/nn1789 – volume: 30 start-page: 892 year: 2020 ident: 10.1016/j.nbd.2023.106022_bb0370 article-title: Cardiolipin in immune signaling and cell death publication-title: Trends Cell Biol. doi: 10.1016/j.tcb.2020.09.004 – volume: 15 year: 2019 ident: 10.1016/j.nbd.2023.106022_bb0470 article-title: TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response publication-title: PLoS Genet. doi: 10.1371/journal.pgen.1007947 – volume: 47 start-page: 142 year: 2013 ident: 10.1016/j.nbd.2023.106022_bb0095 article-title: Hemangioma of the semimembranosus muscle in a patient with late-onset glycogenosis II publication-title: Muscle Nerve doi: 10.1002/mus.23533 – volume: 13 start-page: 489 year: 2019 ident: 10.1016/j.nbd.2023.106022_bb0170 article-title: Mitoautophagy: a unique self-destructive path mitochondria of upper motor neurons with TDP-43 pathology take, very early in ALS publication-title: Front. Cell. Neurosci. doi: 10.3389/fncel.2019.00489 – volume: 5 start-page: 1319 year: 2015 ident: 10.1016/j.nbd.2023.106022_bb0295 article-title: High-resolution Respirometry for simultaneous measurement of oxygen and hydrogen peroxide fluxes in Permeabilized cells, tissue homogenate and isolated mitochondria publication-title: Biomolecules. doi: 10.3390/biom5031319 – volume: 14 year: 2021 ident: 10.1016/j.nbd.2023.106022_bb0390 article-title: Mitochondrial function in development and disease publication-title: Dis. Model. Mech. doi: 10.1242/dmm.048912 – volume: 22 start-page: 4706 year: 2013 ident: 10.1016/j.nbd.2023.106022_bb0460 article-title: The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons publication-title: Hum. Mol. Genet. doi: 10.1093/hmg/ddt319 – volume: 126 start-page: 307 year: 2013 ident: 10.1016/j.nbd.2023.106022_bb0155 article-title: Hereditary spastic paraplegia: clinico-pathologic features and emerging molecular mechanisms publication-title: Acta Neuropathol. doi: 10.1007/s00401-013-1115-8 – volume: 7 start-page: 241 year: 2006 ident: 10.1016/j.nbd.2023.106022_bb0520 article-title: Investigation of the therapeutic effects of edaravone, a free radical scavenger, on amyotrophic lateral sclerosis (Phase II study) publication-title: Amyotroph. Lateral Scler. doi: 10.1080/17482960600881870 – volume: 93 start-page: 871 issue: 8 year: 2022 ident: 10.1016/j.nbd.2023.106022_bb0355 article-title: Effect of sodium phenylbutyrate/taurursodiol on tracheostomy/ventilation-free survival and hospitalisation in amyotrophic lateral sclerosis: long-term results from the CENTAUR trial publication-title: J. Neurol. Neurosurg. Psychiatry doi: 10.1136/jnnp-2022-329024 – volume: 1862 year: 2020 ident: 10.1016/j.nbd.2023.106022_bb0075 article-title: Tau-induced mitochondrial membrane perturbation is dependent upon cardiolipin publication-title: Biochim. Biophys. Acta Biomembr. doi: 10.1016/j.bbamem.2019.183064 – volume: 30 start-page: 734 year: 2008 ident: 10.1016/j.nbd.2023.106022_bb0515 article-title: Edaravone inhibits the induction of iNOS gene expression at transcriptional and posttranscriptional steps in murine macrophages publication-title: Shock. doi: 10.1097/SHK.0b013e318173ea0b – volume: 11 year: 2021 ident: 10.1016/j.nbd.2023.106022_bb0140 article-title: The dying forward hypothesis of ALS: tracing its history publication-title: Brain Sci. doi: 10.3390/brainsci11030300 – volume: 129 start-page: 2436 year: 2006 ident: 10.1016/j.nbd.2023.106022_bb0455 article-title: Novel threshold tracking techniques suggest that cortical hyperexcitability is an early feature of motor neuron disease publication-title: Brain. doi: 10.1093/brain/awl172 – volume: 106 start-page: 18809 year: 2009 ident: 10.1016/j.nbd.2023.106022_bb0475 article-title: TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration publication-title: Proc. Natl. Acad. Sci. U. S. A. doi: 10.1073/pnas.0908767106 – volume: 292 start-page: C33 year: 2007 ident: 10.1016/j.nbd.2023.106022_bb0085 article-title: Role of cardiolipin alterations in mitochondrial dysfunction and disease publication-title: Am. J. Phys. Cell Phys. – volume: 33 start-page: 7890 year: 2013 ident: 10.1016/j.nbd.2023.106022_bb0510 article-title: eGFP expression under UCHL1 promoter genetically labels corticospinal motor neurons and a subpopulation of degeneration-resistant spinal motor neurons in an ALS mouse model publication-title: J. Neurosci. doi: 10.1523/JNEUROSCI.2787-12.2013 – volume: 13 start-page: 89 year: 2015 ident: 10.1016/j.nbd.2023.106022_bb0255 article-title: Structure and function of mitochondrial membrane protein complexes publication-title: BMC Biol. doi: 10.1186/s12915-015-0201-x – volume: 334 start-page: 35 year: 2015 ident: 10.1016/j.nbd.2023.106022_bb0275 article-title: Regulation of mitochondrial transport in neurons publication-title: Exp. Cell Res. doi: 10.1016/j.yexcr.2015.01.004 – volume: 66 start-page: 136 year: 2022 ident: 10.1016/j.nbd.2023.106022_bb0360 article-title: Survival analyses from the CENTAUR trial in amyotrophic lateral sclerosis: evaluating the impact of treatment crossover on outcomes publication-title: Muscle Nerve doi: 10.1002/mus.27569 – volume: 11 year: 2021 ident: 10.1016/j.nbd.2023.106022_bb0305 article-title: Upper and lower motor neuron degenerations are Somatotopically related and temporally ordered in the Sod1 mouse model of amyotrophic lateral sclerosis publication-title: Brain Sci. doi: 10.3390/brainsci11030369 – volume: 181 start-page: 168 year: 2020 ident: 10.1016/j.nbd.2023.106022_bb0395 article-title: Mitochondrial diseases: Hope for the future publication-title: Cell. doi: 10.1016/j.cell.2020.02.051 – volume: 23 start-page: 696 year: 2018 ident: 10.1016/j.nbd.2023.106022_bb0125 article-title: Incorporating upper motor neuron health in ALS drug discovery publication-title: Drug Discov. Today doi: 10.1016/j.drudis.2018.01.027 – volume: 14 start-page: 129 year: 2017 ident: 10.1016/j.nbd.2023.106022_bb0235 article-title: Evidence for an early innate immune response in the motor cortex of ALS publication-title: J. Neuroinflammation doi: 10.1186/s12974-017-0896-4 – volume: 61 start-page: 427 year: 2007 ident: 10.1016/j.nbd.2023.106022_bb0285 article-title: Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations publication-title: Ann. Neurol. doi: 10.1002/ana.21147 – volume: 10 start-page: 1 year: 1885 ident: 10.1016/j.nbd.2023.106022_bb0080 article-title: Deux nouveaux cas de sclerose latérale amyotrophique suivis d’autopsie publication-title: Archives De Neurologie. – volume: 16 start-page: 196 year: 2019 ident: 10.1016/j.nbd.2023.106022_bb0240 article-title: MCP1-CCR2 and neuroinflammation in the ALS motor cortex with TDP-43 pathology publication-title: J. Neuroinflammation doi: 10.1186/s12974-019-1589-y – volume: 1880 start-page: 211 year: 2019 ident: 10.1016/j.nbd.2023.106022_bb0020 article-title: Improved Electron microscopy fixation methods for tracking autophagy-associated membranes in cultured mammalian cells publication-title: Methods Mol. Biol. doi: 10.1007/978-1-4939-8873-0_13 – volume: 18 start-page: 257 year: 2017 ident: 10.1016/j.nbd.2023.106022_bb0100 article-title: Recent advances in mitochondrial disease publication-title: Annu. Rev. Genomics Hum. Genet. doi: 10.1146/annurev-genom-091416-035426 – volume: 43 start-page: 470 year: 2011 ident: 10.1016/j.nbd.2023.106022_bb0110 article-title: The “dying-back” phenomenon of motor neurons in ALS publication-title: J. Mol. Neurosci. doi: 10.1007/s12031-010-9467-1 – volume: 30 start-page: 10851 year: 2010 ident: 10.1016/j.nbd.2023.106022_bb0505 article-title: Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice publication-title: J. Neurosci. doi: 10.1523/JNEUROSCI.1630-10.2010 |
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| Title | SBT-272 improves TDP-43 pathology in ALS upper motor neurons by modulating mitochondrial integrity, motility, and function |
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