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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Veröffentlicht in:	Neurobiology of disease Jg. 178; S. 106022
Hauptverfasser:	Gautam, Mukesh, Genç, Barış, Helmold, Benjamin, Ahrens, Angela, Kuka, Janis, Makrecka-Kuka, Marina, Günay, Aksu, Koçak, Nuran, Aguilar-Wickings, Izaak R., Keefe, Dennis, Zheng, Guozhu, Swaminathan, Suchitra, Redmon, Martin, Zariwala, Hatim A., Özdinler, P. Hande
Format:	Journal Article
Sprache:	Englisch
Veröffentlicht:	United States Elsevier Inc 01.03.2023 Elsevier
Schlagworte:	Amyotrophic Lateral Sclerosis - metabolism Animals ATP Cardiolipin DNA-Binding Proteins - metabolism Electron transport chain Humans Mice Mitochondria - pathology Motor neuron disease Motor Neurons - pathology Proteinopathy SBT-272 TDP-43 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
ISSN:	0969-9961, 1095-953X, 1095-953X
Online-Zugang:	Volltext
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