Gpnmb Is a Potential Marker for the Visceral Pathology in Niemann-Pick Type C Disease
Impaired function of NPC1 or NPC2 lysosomal proteins leads to the intracellular accumulation of unesterified cholesterol, the primary defect underlying Niemann-Pick type C (NPC) disease. In addition, glycosphingolipids (GSLs) accumulate in lysosomes as well. Intralysosomal lipid accumulation trigger...
Gespeichert in:
| Veröffentlicht in: | PloS one Jg. 11; H. 1; S. e0147208 |
|---|---|
| Hauptverfasser: | , , , , , , , , , , |
| Format: | Journal Article |
| Sprache: | Englisch |
| Veröffentlicht: |
United States
Public Library of Science
15.01.2016
Public Library of Science (PLoS) |
| Schlagworte: | |
| ISSN: | 1932-6203, 1932-6203 |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| Abstract | Impaired function of NPC1 or NPC2 lysosomal proteins leads to the intracellular accumulation of unesterified cholesterol, the primary defect underlying Niemann-Pick type C (NPC) disease. In addition, glycosphingolipids (GSLs) accumulate in lysosomes as well. Intralysosomal lipid accumulation triggers the activation of a set of genes, including potential biomarkers. Transcript levels of Gpnmb have been shown to be elevated in various tissues of an NPC mouse model. We speculated that Gpnmb could serve as a marker for visceral lipid accumulation in NPC disease. We report that Gpnmb expression is increased at protein level in macrophages in the viscera of Npc1nih/nih mice. Interestingly, soluble Gpnmb was also found to be increased in murine and NPC patient plasma. Exposure of RAW264.7 macrophages to the NPC-phenotype-inducing drug U18666A also upregulated Gpnmb expression. Inhibition of GSL synthesis with the glucosylceramide synthase (GCS) inhibitor N-butyl-1-deoxynojirimycin prevented U18666A-induced Gpnmb induction and secretion. In summary, we show that Gpnmb is upregulated in NPC mice and patients, most likely due to GSL accumulation. |
|---|---|
| AbstractList | Impaired function of NPC1 or NPC2 lysosomal proteins leads to the intracellular accumulation of unesterified cholesterol, the primary defect underlying Niemann-Pick type C (NPC) disease. In addition, glycosphingolipids (GSLs) accumulate in lysosomes as well. Intralysosomal lipid accumulation triggers the activation of a set of genes, including potential biomarkers. Transcript levels of Gpnmb have been shown to be elevated in various tissues of an NPC mouse model. We speculated that Gpnmb could serve as a marker for visceral lipid accumulation in NPC disease. We report that Gpnmb expression is increased at protein level in macrophages in the viscera of Npc1nih/nih mice. Interestingly, soluble Gpnmb was also found to be increased in murine and NPC patient plasma. Exposure of RAW264.7 macrophages to the NPC-phenotype-inducing drug U18666A also upregulated Gpnmb expression. Inhibition of GSL synthesis with the glucosylceramide synthase (GCS) inhibitor N-butyl-1-deoxynojirimycin prevented U18666A-induced Gpnmb induction and secretion. In summary, we show that Gpnmb is upregulated in NPC mice and patients, most likely due to GSL accumulation. Impaired function of NPC1 or NPC2 lysosomal proteins leads to the intracellular accumulation of unesterified cholesterol, the primary defect underlying Niemann-Pick type C (NPC) disease. In addition, glycosphingolipids (GSLs) accumulate in lysosomes as well. Intralysosomal lipid accumulation triggers the activation of a set of genes, including potential biomarkers. Transcript levels of Gpnmb have been shown to be elevated in various tissues of an NPC mouse model. We speculated that Gpnmb could serve as a marker for visceral lipid accumulation in NPC disease. We report that Gpnmb expression is increased at protein level in macrophages in the viscera of Npc1.sup.nih/nih mice. Interestingly, soluble Gpnmb was also found to be increased in murine and NPC patient plasma. Exposure of RAW264.7 macrophages to the NPC-phenotype-inducing drug U18666A also upregulated Gpnmb expression. Inhibition of GSL synthesis with the glucosylceramide synthase (GCS) inhibitor N-butyl-1-deoxynojirimycin prevented U18666A-induced Gpnmb induction and secretion. In summary, we show that Gpnmb is upregulated in NPC mice and patients, most likely due to GSL accumulation. Impaired function of NPC1 or NPC2 lysosomal proteins leads to the intracellular accumulation of unesterified cholesterol, the primary defect underlying Niemann-Pick type C (NPC) disease. In addition, glycosphingolipids (GSLs) accumulate in lysosomes as well. Intralysosomal lipid accumulation triggers the activation of a set of genes, including potential biomarkers. Transcript levels of Gpnmb have been shown to be elevated in various tissues of an NPC mouse model. We speculated that Gpnmb could serve as a marker for visceral lipid accumulation in NPC disease. We report that Gpnmb expression is increased at protein level in macrophages in the viscera of Npc1 nih/nih mice. Interestingly, soluble Gpnmb was also found to be increased in murine and NPC patient plasma. Exposure of RAW264.7 macrophages to the NPC-phenotype-inducing drug U18666A also upregulated Gpnmb expression. Inhibition of GSL synthesis with the glucosylceramide synthase (GCS) inhibitor N-butyl-1-deoxynojirimycin prevented U18666A-induced Gpnmb induction and secretion. In summary, we show that Gpnmb is upregulated in NPC mice and patients, most likely due to GSL accumulation. |
| Audience | Academic |
| Author | Gabriel, Tanit L. Ottenhoff, Roelof Irún, Pilar Claessen, Nike Alfonso, Pilar Aten, Jan Giraldo, Pilar van Eijk, Marco van Roomen, Cindy P. A. A. Marques, André R. A. Aerts, Johannes M. F. G. |
| AuthorAffiliation | 4 Department of Biochemistry, Leiden Institute of Chemistry, Leiden University, 2300 RA, Leiden, The Netherlands University Hospital S. Maria della Misericordia, Udine, ITALY 3 Centro de Investigación Biomédica en Red de Enfermedades Raras, Unidad de Investigación Traslacional, Zaragoza, Spain 2 Department of Pathology, Academic Medical Center, 1105 AZ, Amsterdam, The Netherlands 1 Department of Medical Biochemistry, Academic Medical Center, 1105 AZ, Amsterdam, The Netherlands |
| AuthorAffiliation_xml | – name: University Hospital S. Maria della Misericordia, Udine, ITALY – name: 4 Department of Biochemistry, Leiden Institute of Chemistry, Leiden University, 2300 RA, Leiden, The Netherlands – name: 2 Department of Pathology, Academic Medical Center, 1105 AZ, Amsterdam, The Netherlands – name: 3 Centro de Investigación Biomédica en Red de Enfermedades Raras, Unidad de Investigación Traslacional, Zaragoza, Spain – name: 1 Department of Medical Biochemistry, Academic Medical Center, 1105 AZ, Amsterdam, The Netherlands |
| Author_xml | – sequence: 1 givenname: André R. A. surname: Marques fullname: Marques, André R. A. – sequence: 2 givenname: Tanit L. surname: Gabriel fullname: Gabriel, Tanit L. – sequence: 3 givenname: Jan surname: Aten fullname: Aten, Jan – sequence: 4 givenname: Cindy P. A. A. surname: van Roomen fullname: van Roomen, Cindy P. A. A. – sequence: 5 givenname: Roelof surname: Ottenhoff fullname: Ottenhoff, Roelof – sequence: 6 givenname: Nike surname: Claessen fullname: Claessen, Nike – sequence: 7 givenname: Pilar surname: Alfonso fullname: Alfonso, Pilar – sequence: 8 givenname: Pilar surname: Irún fullname: Irún, Pilar – sequence: 9 givenname: Pilar surname: Giraldo fullname: Giraldo, Pilar – sequence: 10 givenname: Johannes M. F. G. surname: Aerts fullname: Aerts, Johannes M. F. G. – sequence: 11 givenname: Marco surname: van Eijk fullname: van Eijk, Marco |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/26771826$$D View this record in MEDLINE/PubMed |
| BookMark | eNqNk11v0zAUhiM0xD7gHyCIhITgosVfsZNdIE0FRqXBKth2azn2SestiUucIPrvcWiKmmlCKBeOjp_3tc8rn-PooHY1RNFzjKaYCvzu1nVNrcrpOpSnCDNBUPooOsIZJRNOED3Y-z-Mjr2_RSihKedPokPChcAp4UfR9fm6rvJ47mMVL1wLdWtVGX9RzR00ceGauF1BfGO9hibUF6pdudItN7Gt468WKlXXk4XVd_HVZg3xLP5gPSgPT6PHhSo9PBvWk-j608er2efJxeX5fHZ2MdEiSdsJBwCTE4w5QwYJbgzBhUmMyIRKNSEmV0WRZSwzOjcFpUBJBhkXGGiuhVD0JHq59V2XzsshES-xSAThhJE0EPMtYZy6levGVqrZSKes_FNwzVKqprW6BKlTwQqghSE0ZQIlCtGMGkQLhrCikAev98NpXV6B0SGsEMrIdLxT25Vcup-SCczShAeDN4NB43504FtZ9cmWparBdf29OUoFT5gI6Kt76MPdDdRShQZsXbhwru5N5RljCFGUot5r-gAVPgOV1eH5FDbUR4K3I0FgWvjVLlXnvZx___b_7OXNmH29x65Ale3Ku7Jrrav9GHyxn_TfiHfvNgCnW0A3zvsGCqltq3qf0JotJUayH5JdaLIfEjkMSRCze-Kd_z9lvwF0IhP2 |
| CitedBy_id | crossref_primary_10_1016_j_bbalip_2025_159657 crossref_primary_10_1038_s41598_025_07415_6 crossref_primary_10_1007_s12035_020_01921_6 crossref_primary_10_3390_ijms22010452 crossref_primary_10_1007_s12031_019_01411_y crossref_primary_10_1007_s12035_023_03798_7 crossref_primary_10_1016_j_bbalip_2019_04_002 crossref_primary_10_3390_ijms22052532 crossref_primary_10_1038_s41598_021_99090_6 crossref_primary_10_26508_lsa_202201881 crossref_primary_10_1016_j_bone_2019_115042 crossref_primary_10_1016_j_bbrc_2022_06_082 crossref_primary_10_3389_fimmu_2018_03089 crossref_primary_10_1016_j_preteyeres_2022_101064 crossref_primary_10_1016_j_pharmthera_2017_05_010 crossref_primary_10_1016_j_ymgme_2023_107631 crossref_primary_10_1002_2211_5463_12078 crossref_primary_10_1186_s12974_019_1532_2 crossref_primary_10_1016_j_nbd_2018_08_013 crossref_primary_10_3390_ijms22084039 crossref_primary_10_1016_j_jlr_2025_100825 crossref_primary_10_1016_j_ymgme_2020_02_002 crossref_primary_10_1038_s41598_021_89800_5 crossref_primary_10_3390_jcm10204796 crossref_primary_10_1038_s41598_024_54272_w crossref_primary_10_1186_s40478_018_0612_3 crossref_primary_10_3390_ijms20010066 crossref_primary_10_1038_s41598_017_06264_2 crossref_primary_10_1038_s41598_020_67389_5 crossref_primary_10_3389_fimmu_2021_674739 crossref_primary_10_1186_s13024_025_00874_5 crossref_primary_10_1186_s40364_023_00448_x crossref_primary_10_1016_j_neulet_2019_03_019 crossref_primary_10_1007_s10048_017_0514_8 crossref_primary_10_3389_fgene_2020_00952 crossref_primary_10_3390_ijms20051152 crossref_primary_10_1016_j_bcmd_2016_12_002 crossref_primary_10_1038_s41598_022_06112_y crossref_primary_10_1186_s12974_023_02965_w crossref_primary_10_1093_hmg_ddab194 crossref_primary_10_1016_j_celrep_2025_115343 crossref_primary_10_1002_2211_5463_13651 crossref_primary_10_1038_s41598_022_10522_3 crossref_primary_10_1371_journal_pone_0182075 |
| Cites_doi | 10.1038/srep00573 10.1074/jbc.273.41.26522 10.1096/fj.10-154757 10.1096/fj.09-151019 10.1016/S0021-9258(18)80136-3 10.1371/journal.pone.0047845 10.1194/jlr.M800415-JLR200 10.1074/jbc.M112.412460 10.1371/journal.pone.0028777 10.1016/j.bbalip.2004.08.005 10.1371/journal.pone.0114669 10.1093/hmg/ddr505 10.1074/jbc.M113.526392 10.1371/journal.pone.0012093 10.1007/s10545-006-0363-3 10.1093/hmg/dds126 10.1016/0005-2760(83)90218-7 10.1038/nature04399 10.1126/science.277.5323.228 10.1021/jo061280p 10.1073/pnas.1003308107 10.1186/s13023-015-0274-1 10.1016/S0021-9258(18)47132-3 10.1007/s10545-006-0318-8 10.1016/0005-2760(94)90156-2 10.1016/S0021-9258(18)64849-5 10.1016/S0022-2275(20)40725-4 10.1186/1750-1172-5-16 10.1093/hmg/dds193 10.4049/jimmunol.177.1.26 10.1194/jlr.M007211 10.1139/o59-099 10.1371/journal.pone.0120194 10.1371/journal.pone.0038520 10.1126/science.277.5323.232 10.1016/j.nbd.2009.07.010 10.1016/S1474-4422(07)70194-1 10.1016/j.febslet.2007.11.036 10.1016/j.ymgme.2013.03.015 10.1016/0005-2760(83)90232-1 10.1126/scitranslmed.3001417 10.1371/journal.pone.0048273 10.1016/S0306-4522(01)00230-5 10.1074/jbc.M313517200 10.1016/j.cmet.2013.11.001 10.2337/db13-1720 10.1172/JCI117084 10.1002/path.2667 10.4049/jimmunol.178.10.6557 |
| ContentType | Journal Article |
| Copyright | COPYRIGHT 2016 Public Library of Science 2016 Marques et al. This is an open access article distributed under the terms of the Creative Commons Attribution License: http://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License. 2016 Marques et al 2016 Marques et al |
| Copyright_xml | – notice: COPYRIGHT 2016 Public Library of Science – notice: 2016 Marques et al. This is an open access article distributed under the terms of the Creative Commons Attribution License: http://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License. – notice: 2016 Marques et al 2016 Marques et al |
| DBID | AAYXX CITATION CGR CUY CVF ECM EIF NPM IOV ISR 3V. 7QG 7QL 7QO 7RV 7SN 7SS 7T5 7TG 7TM 7U9 7X2 7X7 7XB 88E 8AO 8C1 8FD 8FE 8FG 8FH 8FI 8FJ 8FK ABJCF ABUWG AEUYN AFKRA ARAPS ATCPS AZQEC BBNVY BENPR BGLVJ BHPHI C1K CCPQU D1I DWQXO FR3 FYUFA GHDGH GNUQQ H94 HCIFZ K9. KB. KB0 KL. L6V LK8 M0K M0S M1P M7N M7P M7S NAPCQ P5Z P62 P64 PATMY PDBOC PHGZM PHGZT PIMPY PJZUB PKEHL PPXIY PQEST PQGLB PQQKQ PQUKI PRINS PTHSS PYCSY RC3 7X8 5PM DOA |
| DOI | 10.1371/journal.pone.0147208 |
| DatabaseName | CrossRef Medline MEDLINE MEDLINE (Ovid) MEDLINE MEDLINE PubMed Gale In Context: Opposing Viewpoints Gale In Context: Science ProQuest Central (Corporate) Animal Behavior Abstracts Bacteriology Abstracts (Microbiology B) Biotechnology Research Abstracts Nursing & Allied Health Database Ecology Abstracts Entomology Abstracts (Full archive) Immunology Abstracts Meteorological & Geoastrophysical Abstracts Nucleic Acids Abstracts Virology and AIDS Abstracts Agricultural Science Collection Health & Medical Collection ProQuest Central (purchase pre-March 2016) Medical Database (Alumni Edition) ProQuest Pharma Collection Public Health Database (ProQuest) Technology Research Database ProQuest SciTech Collection ProQuest Technology Collection ProQuest Natural Science Collection Hospital Premium Collection Hospital Premium Collection (Alumni Edition) ProQuest Central (Alumni) (purchase pre-March 2016) Materials Science & Engineering Collection ProQuest Central (Alumni) ProQuest One Sustainability ProQuest Central UK/Ireland Advanced Technologies & Computer Science Collection Agricultural & Environmental Science Collection ProQuest Central Essentials Biological Science Collection ProQuest Central Technology Collection Natural Science Collection Environmental Sciences and Pollution Management ProQuest One ProQuest Materials Science Collection ProQuest Central Engineering Research Database Health Research Premium Collection Health Research Premium Collection (Alumni) ProQuest Central Student AIDS and Cancer Research Abstracts SciTech Collection (ProQuest) ProQuest Health & Medical Complete (Alumni) Materials Science Database Nursing & Allied Health Database (Alumni Edition) Meteorological & Geoastrophysical Abstracts - Academic ProQuest Engineering Collection ProQuest Biological Science Collection Agricultural Science Database ProQuest Health & Medical Collection Medical Database Algology Mycology and Protozoology Abstracts (Microbiology C) Biological Science Database (ProQuest) Engineering Database Nursing & Allied Health Premium Advanced Technologies & Aerospace Database ProQuest Advanced Technologies & Aerospace Collection Biotechnology and BioEngineering Abstracts Environmental Science Database Materials Science Collection ProQuest Central Premium ProQuest One Academic (New) Publicly Available Content Database ProQuest Health & Medical Research Collection ProQuest One Academic Middle East (New) ProQuest One Health & Nursing ProQuest One Academic Eastern Edition (DO NOT USE) ProQuest One Applied & Life Sciences ProQuest One Academic (retired) ProQuest One Academic UKI Edition ProQuest Central China Engineering Collection Environmental Science Collection Genetics Abstracts MEDLINE - Academic PubMed Central (Full Participant titles) DOAJ Directory of Open Access Journals |
| DatabaseTitle | CrossRef MEDLINE Medline Complete MEDLINE with Full Text PubMed MEDLINE (Ovid) Agricultural Science Database Publicly Available Content Database ProQuest Central Student ProQuest Advanced Technologies & Aerospace Collection ProQuest Central Essentials Nucleic Acids Abstracts SciTech Premium Collection ProQuest Central China Environmental Sciences and Pollution Management ProQuest One Applied & Life Sciences ProQuest One Sustainability Health Research Premium Collection Meteorological & Geoastrophysical Abstracts Natural Science Collection Health & Medical Research Collection Biological Science Collection ProQuest Central (New) ProQuest Medical Library (Alumni) Engineering Collection Advanced Technologies & Aerospace Collection Engineering Database Virology and AIDS Abstracts ProQuest Biological Science Collection ProQuest One Academic Eastern Edition Agricultural Science Collection ProQuest Hospital Collection ProQuest Technology Collection Health Research Premium Collection (Alumni) Biological Science Database Ecology Abstracts ProQuest Hospital Collection (Alumni) Biotechnology and BioEngineering Abstracts Environmental Science Collection Entomology Abstracts Nursing & Allied Health Premium ProQuest Health & Medical Complete ProQuest One Academic UKI Edition Environmental Science Database ProQuest Nursing & Allied Health Source (Alumni) Engineering Research Database ProQuest One Academic Meteorological & Geoastrophysical Abstracts - Academic ProQuest One Academic (New) Technology Collection Technology Research Database ProQuest One Academic Middle East (New) Materials Science Collection ProQuest Health & Medical Complete (Alumni) ProQuest Central (Alumni Edition) ProQuest One Community College ProQuest One Health & Nursing ProQuest Natural Science Collection ProQuest Pharma Collection ProQuest Central ProQuest Health & Medical Research Collection Genetics Abstracts ProQuest Engineering Collection Biotechnology Research Abstracts Health and Medicine Complete (Alumni Edition) ProQuest Central Korea Bacteriology Abstracts (Microbiology B) Algology Mycology and Protozoology Abstracts (Microbiology C) Agricultural & Environmental Science Collection AIDS and Cancer Research Abstracts Materials Science Database ProQuest Materials Science Collection ProQuest Public Health ProQuest Nursing & Allied Health Source ProQuest SciTech Collection Advanced Technologies & Aerospace Database ProQuest Medical Library Animal Behavior Abstracts Materials Science & Engineering Collection Immunology Abstracts ProQuest Central (Alumni) MEDLINE - Academic |
| DatabaseTitleList | MEDLINE Agricultural Science Database MEDLINE - Academic |
| Database_xml | – sequence: 1 dbid: DOA name: DOAJ Directory of Open Access Journals url: https://www.doaj.org/ sourceTypes: Open Website – sequence: 2 dbid: NPM name: PubMed url: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed sourceTypes: Index Database – sequence: 3 dbid: PIMPY name: Publicly Available Content Database url: http://search.proquest.com/publiccontent sourceTypes: Aggregation Database |
| DeliveryMethod | fulltext_linktorsrc |
| Discipline | Sciences (General) |
| DocumentTitleAlternate | Gpnmb Is a Marker for NPC |
| EISSN | 1932-6203 |
| EndPage | e0147208 |
| ExternalDocumentID | 1757262428 oai_doaj_org_article_c874fe3fd2384705a0393d03f401a3eb PMC4714856 3923035531 A440030807 26771826 10_1371_journal_pone_0147208 |
| Genre | Research Support, Non-U.S. Gov't Journal Article |
| GeographicLocations | United States Netherlands Spain |
| GeographicLocations_xml | – name: United States – name: Netherlands – name: Spain |
| GroupedDBID | --- 123 29O 2WC 53G 5VS 7RV 7X2 7X7 7XC 88E 8AO 8C1 8CJ 8FE 8FG 8FH 8FI 8FJ A8Z AAFWJ AAUCC AAWOE AAYXX ABDBF ABIVO ABJCF ABUWG ACCTH ACGFO ACIHN ACIWK ACPRK ACUHS ADBBV ADRAZ AEAQA AENEX AEUYN AFFHD AFKRA AFPKN AFRAH AHMBA ALMA_UNASSIGNED_HOLDINGS AOIJS APEBS ARAPS ATCPS BAIFH BAWUL BBNVY BBTPI BCNDV BENPR BGLVJ BHPHI BKEYQ BPHCQ BVXVI BWKFM CCPQU CITATION CS3 D1I D1J D1K DIK DU5 E3Z EAP EAS EBD EMOBN ESX EX3 F5P FPL FYUFA GROUPED_DOAJ GX1 HCIFZ HH5 HMCUK HYE IAO IEA IGS IHR IHW INH INR IOV IPY ISE ISR ITC K6- KB. KQ8 L6V LK5 LK8 M0K M1P M48 M7P M7R M7S M~E NAPCQ O5R O5S OK1 OVT P2P P62 PATMY PDBOC PHGZM PHGZT PIMPY PJZUB PPXIY PQGLB PQQKQ PROAC PSQYO PTHSS PV9 PYCSY RNS RPM RZL SV3 TR2 UKHRP WOQ WOW ~02 ~KM ALIPV BBORY CGR CUY CVF ECM EIF IPNFZ NPM RIG 3V. 7QG 7QL 7QO 7SN 7SS 7T5 7TG 7TM 7U9 7XB 8FD 8FK AZQEC C1K DWQXO ESTFP FR3 GNUQQ H94 K9. KL. M7N P64 PKEHL PQEST PQUKI PRINS RC3 7X8 5PM - 02 AAPBV ABPTK ADACO BBAFP KM |
| ID | FETCH-LOGICAL-c758t-6eeedb211640d076dd21fd5d797a8c22dbaff9949dcbdf33e329e9671e3bc77a3 |
| IEDL.DBID | FPL |
| ISICitedReferencesCount | 46 |
| ISICitedReferencesURI | http://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=Summon&SrcAuth=ProQuest&DestLinkType=CitingArticles&DestApp=WOS_CPL&KeyUT=000368628300072&url=https%3A%2F%2Fcvtisr.summon.serialssolutions.com%2F%23%21%2Fsearch%3Fho%3Df%26include.ft.matches%3Dt%26l%3Dnull%26q%3D |
| ISSN | 1932-6203 |
| IngestDate | Fri Nov 26 17:12:43 EST 2021 Fri Oct 03 12:41:33 EDT 2025 Tue Nov 04 01:57:06 EST 2025 Sun Nov 09 13:25:36 EST 2025 Tue Oct 07 07:28:02 EDT 2025 Sat Nov 29 13:17:19 EST 2025 Sat Nov 29 10:01:45 EST 2025 Wed Nov 26 10:14:16 EST 2025 Wed Nov 26 10:27:23 EST 2025 Thu May 22 21:19:44 EDT 2025 Thu Apr 03 07:10:15 EDT 2025 Sat Nov 29 04:00:12 EST 2025 Tue Nov 18 20:54:38 EST 2025 |
| IsDoiOpenAccess | true |
| IsOpenAccess | true |
| IsPeerReviewed | true |
| IsScholarly | true |
| Issue | 1 |
| Language | English |
| License | This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Creative Commons Attribution License |
| LinkModel | DirectLink |
| MergedId | FETCHMERGED-LOGICAL-c758t-6eeedb211640d076dd21fd5d797a8c22dbaff9949dcbdf33e329e9671e3bc77a3 |
| Notes | ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 14 content type line 23 Conceived and designed the experiments: ARAM TLG JMFGA MvE. Performed the experiments: ARAM TLG JA CPAAvR RO NC PI PA PG MvE. Analyzed the data: ARAM TLG JA MvE. Contributed reagents/materials/analysis tools: PI PA PG. Wrote the paper: ARAM TLG JA JMFGA MvE. Competing Interests: The authors have declared that no competing interests exist. |
| OpenAccessLink | http://dx.doi.org/10.1371/journal.pone.0147208 |
| PMID | 26771826 |
| PQID | 1757262428 |
| PQPubID | 1436336 |
| ParticipantIDs | plos_journals_1757262428 doaj_primary_oai_doaj_org_article_c874fe3fd2384705a0393d03f401a3eb pubmedcentral_primary_oai_pubmedcentral_nih_gov_4714856 proquest_miscellaneous_1760876547 proquest_journals_1757262428 gale_infotracmisc_A440030807 gale_infotracacademiconefile_A440030807 gale_incontextgauss_ISR_A440030807 gale_incontextgauss_IOV_A440030807 gale_healthsolutions_A440030807 pubmed_primary_26771826 crossref_citationtrail_10_1371_journal_pone_0147208 crossref_primary_10_1371_journal_pone_0147208 |
| PublicationCentury | 2000 |
| PublicationDate | 2016-01-15 |
| PublicationDateYYYYMMDD | 2016-01-15 |
| PublicationDate_xml | – month: 01 year: 2016 text: 2016-01-15 day: 15 |
| PublicationDecade | 2010 |
| PublicationPlace | United States |
| PublicationPlace_xml | – name: United States – name: San Francisco – name: San Francisco, CA USA |
| PublicationTitle | PloS one |
| PublicationTitleAlternate | PLoS One |
| PublicationYear | 2016 |
| Publisher | Public Library of Science Public Library of Science (PLoS) |
| Publisher_xml | – name: Public Library of Science – name: Public Library of Science (PLoS) |
| References | FD Porter (ref17) 2010; 2 DC German (ref37) 2001; 105 X Xu (ref51) 2013; 18 Y Sagiv (ref16) 2006; 177 A Amritraj (ref38) 2013; 288 R Salvioli (ref6) 2004; 279 MD Morris (ref9) 1982; 108 NL Sayre (ref13) 2010; 51 L Liscum (ref40) 1989; 264 ED Carstea (ref2) 1997; 277 H Gold (ref31) 2012; 59 FM Platt (ref42) 1994; 269 SM Cologna (ref24) 2013 VM Ripoll (ref39) 2007; 178 MC Vázquez (ref10) 2011; 6 T Wennekes (ref43) 2007; 72 FR Maxfield (ref15) 2005; 438 HS Overkleeft (ref44) 1998; 273 OJ De Boer (ref30) 2010; 220 RWD Welford (ref21) 2014; 9 J Folch (ref33) 1957; 226 VM Rimkunas (ref12) 2009; 50 T Hoashi (ref27) 2010; 24 M Ries (ref45) 2006; 29 PG Pentchev (ref3) 2004; 1685 H Furochi (ref26) 2007; 581 MT Vanier (ref4) 1983; 750 SM Cologna (ref23) 2012; 7 H Tanaka (ref28) 2012; 2 SK Loftus (ref8) 1997; 277 PK Mistry (ref48) 2010; 107 B Li (ref46) 2010; 24 E Lombardo (ref52) 2012; 7 H Zigdon (ref49) 2015; 10 TL Gabriel (ref35) 2014; 63 MS Alam (ref18) 2012; 7 AS Härmälä (ref41) 1994; 1211 RG Boot (ref47) 2006; 29 ME Lopez (ref11) 2012; 21 A-K Giese (ref22) 2015; 10 JG Heider (ref34) 1978; 19 MS Alam (ref19) 2014; 289 RA Maue (ref29) 2012; 21 CE Hollak (ref36) 1994; 93 DJ Sillence (ref50) 2013; 109 EG Bligh (ref32) 1959; 37 CVM Cluzeau (ref20) 2012; 21 MC Patterson (ref7) 2007; 6 AAN Rose (ref25) 2010; 5 MT Vanier (ref1) 2010; 5 GT Besley (ref5) 1983; 752 D Smith (ref14) 2009; 36 |
| References_xml | – volume: 2 start-page: 1 year: 2012 ident: ref28 article-title: The potential of GPNMB as novel neuroprotective factor in amyotrophic lateral sclerosis publication-title: Sci Rep doi: 10.1038/srep00573 – volume: 273 start-page: 26522 year: 1998 ident: ref44 article-title: Generation of specific deoxynojirimycin-type inhibitors of the non-lysosomal glucosylceramidase publication-title: J Biol Chem doi: 10.1074/jbc.273.41.26522 – volume: 24 start-page: 4767 year: 2010 ident: ref46 article-title: The melanoma-associated transmembrane glycoprotein Gpnmb controls trafficking of cellular debris for degradation and is essential for tissue repair publication-title: FASEB J doi: 10.1096/fj.10-154757 – volume: 24 start-page: 1616 year: 2010 ident: ref27 article-title: Glycoprotein nonmetastatic melanoma protein b, a melanocytic cell marker, is a melanosome-specific and proteolytically released protein publication-title: FASEB J doi: 10.1096/fj.09-151019 – volume: 264 start-page: 11796 year: 1989 ident: ref40 article-title: The intracellular transport of low density lipoprotein-derived cholesterol is inhibited in Chinese hamster ovary cells cultured with 3-beta-[2-(diethylamino)ethoxy]androst-5-en-17-one publication-title: J Biol Chem doi: 10.1016/S0021-9258(18)80136-3 – volume: 7 start-page: e47845 year: 2012 ident: ref23 article-title: Quantitative proteomic analysis of niemann-pick disease, type c1 cerebellum identifies protein biomarkers and provides pathological insight publication-title: PLoS One doi: 10.1371/journal.pone.0047845 – volume: 108 start-page: 140 year: 1982 ident: ref9 article-title: Lysosome lipid storage disorder in NCTR-BALB/c mice. I. Description of the disease and genetics publication-title: Am J Pathol – volume: 50 start-page: 327 year: 2009 ident: ref12 article-title: TNF-{alpha} plays a role in hepatocyte apoptosis in Niemann-Pick type C liver disease publication-title: J Lipid Res doi: 10.1194/jlr.M800415-JLR200 – volume: 288 start-page: 3136 year: 2013 ident: ref38 article-title: Role of cathepsin D in U18666A-induced neuronal cell death: potential implication in Niemann Pick Type C disease pathogenesis publication-title: J Biol Chem doi: 10.1074/jbc.M112.412460 – volume: 6 start-page: e28777 year: 2011 ident: ref10 article-title: Alteration of gene expression profile in Niemann-Pick type C mice correlates with tissue damage and oxidative stress publication-title: PLoS One doi: 10.1371/journal.pone.0028777 – volume: 1685 start-page: 3 year: 2004 ident: ref3 article-title: Niemann-Pick C research from mouse to gene publication-title: Biochim Biophys Acta doi: 10.1016/j.bbalip.2004.08.005 – volume: 9 start-page: e114669 year: 2014 ident: ref21 article-title: Plasma lysosphingomyelin demonstrates great potential as a diagnostic biomarker for Niemann-Pick disease type C in a retrospective study publication-title: PLoS One doi: 10.1371/journal.pone.0114669 – year: 2013 ident: ref24 article-title: Human and mouse neuroinflammation markers in Niemann-Pick disease, type C1 publication-title: J Inherit Metab Dis – volume: 21 start-page: 730 year: 2012 ident: ref29 article-title: A novel mouse model of Niemann-Pick type C disease carrying a D1005G-Npc1 mutation comparable to commonly observed human mutations publication-title: Hum Mol Genet doi: 10.1093/hmg/ddr505 – volume: 289 start-page: 8051 year: 2014 ident: ref19 article-title: Plasma signature of neurological disease in the monogenetic disorder Niemann-Pick Type C publication-title: J Biol Chem doi: 10.1074/jbc.M113.526392 – volume: 5 start-page: e12093 year: 2010 ident: ref25 article-title: ADAM10 releases a soluble form of the GPNMB/Osteoactivin extracellular domain with angiogenic properties publication-title: PLoS One doi: 10.1371/journal.pone.0012093 – volume: 29 start-page: 647 year: 2006 ident: ref45 article-title: Critical assessment of chitotriosidase analysis in the rational laboratory diagnosis of children with Gaucher disease and Niemann-Pick disease type A/B and C publication-title: J Inherit Metab Dis doi: 10.1007/s10545-006-0363-3 – volume: 21 start-page: 2946 year: 2012 ident: ref11 article-title: Neuronal and epithelial cell rescue resolves chronic systemic inflammation in the lipid storage disorder Niemann-Pick C publication-title: Hum Mol Genet doi: 10.1093/hmg/dds126 – volume: 750 start-page: 178 year: 1983 ident: ref4 article-title: Biochemical studies in Niemann-Pick disease. I. Major sphingolipids of liver and spleen publication-title: Biochim Biophys Acta doi: 10.1016/0005-2760(83)90218-7 – volume: 438 start-page: 612 year: 2005 ident: ref15 article-title: Role of cholesterol and lipid organization in disease publication-title: Nature doi: 10.1038/nature04399 – volume: 277 start-page: 228 year: 1997 ident: ref2 article-title: Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis publication-title: Science doi: 10.1126/science.277.5323.228 – volume: 72 start-page: 1088 year: 2007 ident: ref43 article-title: Development of adamantan-1-yl-methoxy-functionalized 1-deoxynojirimycin derivatives as selective inhibitors of glucosylceramide metabolism in man publication-title: J Org Chem doi: 10.1021/jo061280p – volume: 107 start-page: 19473 year: 2010 ident: ref48 article-title: Glucocerebrosidase gene-deficient mouse recapitulates Gaucher disease displaying cellular and molecular dysregulation beyond the macrophage publication-title: Proc Natl Acad Sci U S A doi: 10.1073/pnas.1003308107 – volume: 10 start-page: 78 year: 2015 ident: ref22 article-title: A novel, highly sensitive and specific biomarker for Niemann-Pick type C1 disease publication-title: Orphanet J Rare Dis doi: 10.1186/s13023-015-0274-1 – volume: 269 start-page: 27108 year: 1994 ident: ref42 article-title: N-butyldeoxygalactonojirimycin inhibits glycolipid biosynthesis but does not affect N-linked oligosaccharide processing publication-title: J Biol Chem doi: 10.1016/S0021-9258(18)47132-3 – volume: 29 start-page: 564 year: 2006 ident: ref47 article-title: CCL18: A urinary marker of Gaucher cell burden in Gaucher patients publication-title: J Inherit Metab Dis doi: 10.1007/s10545-006-0318-8 – volume: 1211 start-page: 317 year: 1994 ident: ref41 article-title: Cholesterol transport from plasma membranes to intracellular membranes is inhibited by 3 beta-[2-(diethylamino)ethoxy]androst-5-en-17-one publication-title: Biochim Biophys Acta doi: 10.1016/0005-2760(94)90156-2 – volume: 226 start-page: 497 year: 1957 ident: ref33 article-title: A simple method for the isolation and purification of total lipides from animal tissues publication-title: J Biol Chem doi: 10.1016/S0021-9258(18)64849-5 – volume: 19 start-page: 514 year: 1978 ident: ref34 article-title: The picomole determination of free and total cholesterol in cells in culture publication-title: J Lipid Res doi: 10.1016/S0022-2275(20)40725-4 – volume: 5 start-page: 1 year: 2010 ident: ref1 article-title: Niemann-Pick disease type C publication-title: Orphanet J Rare Dis doi: 10.1186/1750-1172-5-16 – volume: 21 start-page: 3632 year: 2012 ident: ref20 article-title: Microarray expression analysis and identification of serum biomarkers for Niemann-Pick disease, type C1 publication-title: Hum Mol Genet doi: 10.1093/hmg/dds193 – volume: 177 start-page: 26 year: 2006 ident: ref16 article-title: Cutting edge: impaired glycosphingolipid trafficking and NKT cell development in mice lacking Niemann-Pick type C1 protein publication-title: J Immunol doi: 10.4049/jimmunol.177.1.26 – volume: 51 start-page: 2372 year: 2010 ident: ref13 article-title: Recovery from liver disease in a Niemann-Pick type C mouse model publication-title: J Lipid Res doi: 10.1194/jlr.M007211 – volume: 37 start-page: 911 year: 1959 ident: ref32 article-title: A rapid method of total lipid extraction and purification publication-title: Can J Biochem Physiol doi: 10.1139/o59-099 – volume: 10 start-page: e0120194 year: 2015 ident: ref49 article-title: Identification of a Biomarker in Cerebrospinal Fluid for Neuronopathic Forms of Gaucher Disease publication-title: PLoS One doi: 10.1371/journal.pone.0120194 – volume: 7 start-page: e38520 year: 2012 ident: ref52 article-title: Correction of liver steatosis by a hydrophobic iminosugar modulating glycosphingolipids metabolism publication-title: PLoS One doi: 10.1371/journal.pone.0038520 – volume: 277 start-page: 232 year: 1997 ident: ref8 article-title: Murine model of Niemann-Pick C disease: mutation in a cholesterol homeostasis gene publication-title: Science doi: 10.1126/science.277.5323.232 – volume: 36 start-page: 242 year: 2009 ident: ref14 article-title: Beneficial effects of anti-inflammatory therapy in a mouse model of Niemann-Pick disease type C1 publication-title: Neurobiol Dis doi: 10.1016/j.nbd.2009.07.010 – volume: 6 start-page: 765 year: 2007 ident: ref7 article-title: Miglustat for treatment of Niemann-Pick C disease: a randomised controlled study publication-title: Lancet Neurol doi: 10.1016/S1474-4422(07)70194-1 – volume: 581 start-page: 5743 year: 2007 ident: ref26 article-title: Osteoactivin fragments produced by ectodomain shedding induce MMP-3 expression via ERK pathway in mouse NIH-3T3 fibroblasts publication-title: FEBS Lett doi: 10.1016/j.febslet.2007.11.036 – volume: 109 start-page: 194 year: 2013 ident: ref50 article-title: Glucosylceramide modulates endolysosomal pH in Gaucher disease publication-title: Mol Genet Metab doi: 10.1016/j.ymgme.2013.03.015 – volume: 752 start-page: 54 year: 1983 ident: ref5 article-title: Studies on sphingomyelinase and beta-glucosidase activities in Niemann-Pick disease variants. Phosphodiesterase activities measured with natural and artificial substrates publication-title: Biochim Biophys Acta doi: 10.1016/0005-2760(83)90232-1 – volume: 2 start-page: 56ra81 year: 2010 ident: ref17 article-title: Cholesterol oxidation products are sensitive and specific blood-based biomarkers for Niemann-Pick C1 disease publication-title: Sci Transl Med doi: 10.1126/scitranslmed.3001417 – volume: 7 start-page: e48273 year: 2012 ident: ref18 article-title: Genomic expression analyses reveal lysosomal, innate immunity proteins, as disease correlates in murine models of a lysosomal storage disorder publication-title: PLoS One doi: 10.1371/journal.pone.0048273 – volume: 105 start-page: 999 year: 2001 ident: ref37 article-title: Degeneration of neurons and glia in the Niemann-Pick C mouse is unrelated to the low-density lipoprotein receptor publication-title: Neuroscience doi: 10.1016/S0306-4522(01)00230-5 – volume: 279 start-page: 17674 year: 2004 ident: ref6 article-title: Glucosylceramidase mass and subcellular localization are modulated by cholesterol in Niemann-Pick disease type C publication-title: J Biol Chem doi: 10.1074/jbc.M313517200 – volume: 18 start-page: 816 year: 2013 ident: ref51 article-title: Obesity activates a program of lysosomal-dependent lipid metabolism in adipose tissue macrophages independently of classic activation publication-title: Cell Metab doi: 10.1016/j.cmet.2013.11.001 – volume: 63 start-page: 3310 year: 2014 ident: ref35 article-title: Lysosomal Stress in Obese Adipose Tissue Macrophages Contributes to MITF-Dependent Gpnmb Induction publication-title: Diabetes doi: 10.2337/db13-1720 – volume: 93 start-page: 1288 year: 1994 ident: ref36 article-title: Marked elevation of plasma chitotriosidase activity. A novel hallmark of Gaucher disease publication-title: J Clin Invest doi: 10.1172/JCI117084 – volume: 59 start-page: 1 year: 2012 ident: ref31 article-title: Quantification of Globotriaosylsphingosine in Plasma and Urine of Fabry Patients by Stable Isotope Ultraperformance Liquid Chromatography-Tandem Mass Spectrometry publication-title: Clin Chem – volume: 220 start-page: 499 year: 2010 ident: ref30 article-title: Differential expression of interleukin-17 family cytokines in intact and complicated human atherosclerotic plaques publication-title: J Pathol doi: 10.1002/path.2667 – volume: 178 start-page: 6557 year: 2007 ident: ref39 article-title: Gpnmb is induced in macrophages by IFN-gamma and lipopolysaccharide and acts as a feedback regulator of proinflammatory responses publication-title: J Immunol doi: 10.4049/jimmunol.178.10.6557 |
| RelatedPersons | Yang, Cindy |
| RelatedPersons_xml | – fullname: Yang, Cindy |
| SSID | ssj0053866 |
| Score | 2.3976212 |
| Snippet | Impaired function of NPC1 or NPC2 lysosomal proteins leads to the intracellular accumulation of unesterified cholesterol, the primary defect underlying... |
| SourceID | plos doaj pubmedcentral proquest gale pubmed crossref |
| SourceType | Open Website Open Access Repository Aggregation Database Index Database Enrichment Source |
| StartPage | e0147208 |
| SubjectTerms | Accumulation Adult Aged Aged, 80 and over Animal tissues Animals Biochemistry Bioindicators Biomarkers Biomarkers - metabolism Brain diseases Care and treatment Cell Line Ceramide glucosyltransferase Cholesterol Cholesterol - metabolism Development and progression Disease Models, Animal Eye Proteins - genetics Eye Proteins - metabolism Female Foam Cells - metabolism Genetic disorders Glycoproteins Glycosphingolipids Glycosphingolipids - metabolism Humans Inborn errors of metabolism Laboratories Lipids Liver Lysosomes Macrophages Male Melanoma Membrane Glycoproteins - genetics Membrane Glycoproteins - metabolism Metabolic disorders Metabolism Mice Middle Aged Mucopolysaccharides Niemann-Pick disease Niemann-Pick Disease, Type C - genetics Niemann-Pick Disease, Type C - metabolism Npc1 protein Pathology Patient outcomes Proteins Risk factors Rodents Secretion Transcription Viscera Yang, Cindy Young Adult |
| SummonAdditionalLinks | – databaseName: DOAJ Directory of Open Access Journals dbid: DOA link: http://cvtisr.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwrV1Lb9NAEF6higMXRHk1EGBBSMDBrR_rneyxFAq9hAho1Zu13kexaJ0oTpD498zYGytGlcqBY7LfrpKZ2d0Ze-Ybxl4rdJLLMk8i6a2KhJY6UsKbCL1nCcYnpdambTYB0-nk_FzNtlp9UU5YRw_cCe7ATEB4l3mLd4uAONdUTGrjzGNgoDNX0ukbg9oEU90ZjLtYylAol0FyEPSyv5jXbh-DAkipneTWRdTy9fen8s7ict5c53L-nTm5dRUd32N3gw_JD7vfvstuufo-2w27tOFvA5X0uwfs9NOivir5ScM1n81XlBmEE6k-xy05uqsc3T9-VjWG8HymV-1Z-JtXNZ9W7krXdTSrzE9O0So_4h-6tzkP2enxx-9Hn6PQSCEyGA6sIunwJiwx1JMitjFIa9PE29yCAj0xaWpL7b1SQllTWp9lLkuVUxISl5UGQGeP2E6Nottj3ONCgErEyVIo_GAw4vGJAiLpiRM7YtlGqoUJLOPU7OKyaF-dAUYbnZAK0kURdDFiUT9r0bFs3IB_TwrrscSR3X6BllMEyyluspwRe0HqLrqC036nF4dCtCw-MYzYqxZBPBk1JeJc6HXTFCdfzv4B9O3rAPQmgPwcxWF0KH7A_0T8WwPkeIDE3W4Gw3tknBupNAW6f5BSkQ8KZbwx2OuHX_bDtCgl19VuviaMJGLCXODqjzv77iWbSgAKQUcMBpY_EP1wpK5-tDTl6PaISS6f_A9dPWV30FNtn30l-ZjtrJZr94zdNr9WVbN83u79P1nbXh4 priority: 102 providerName: Directory of Open Access Journals – databaseName: Nursing & Allied Health Database dbid: 7RV link: http://cvtisr.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwpV1Lb9QwELZg4cAFKI82UMAgJOCQNk97fUKlUOhlWRW66i1y_CgRbbJsdpH498w43tCgCpA4Jh5byXg8nrFnviHkuQAjuSzzOGRWizCTTIYisyoE65lxZeNSSuWKTfDJZHxyIqb-wK31YZVrnegUtW4UnpHvwjbHE0xmGL-efwuxahTervoSGlfJtRhtY5BnfjRba2JYy4z5dLmUx7t-dnbmTW12wDXgCRaVvLAdOdT-XjeP5mdNe5nh-Xv85IUN6eDW__7KbXLTm6J0r5OdDXLF1HfIhl_sLX3pEalf3SXH7-f1eUkPWyrptFligBF0xDQfs6Bg9VKwIumsahXS06lcOpX6g1Y1nVTmXNZ1OK3UV4pOL92nb7tLoXvk-ODd5_0Poa_HECrwKpYhM7ChluAxsizSEWdaJ7HVueaCy7FKEl1Ka4XIhFaltmlq0kQYwXhs0lJxLtP7ZFQD77cItTAQB1mAziwT8KDAcbKx4Ij1E8U6IOl6WgrlwcqxZsZZ4W7gODgtHZMKnMzCT2ZAwr7XvAPr-Av9G5zxnhahtt2LZnFa-JVbqDHPrEmtBuMm41EuMZtZR6kFz1SmpgzIE5SXostb7RVGsZdlDgwo4gF55igQbqPGeJ5TuWrb4vDj7B-IPh0NiF54ItsAO5T0ORTwTwjjNaDcHlCC0lCD5i2U7jVX2uKXTELPtdRe3vy0b8ZBMUavNs0KaRjiG-YZjL7ZLZCeswnjHD3ZgPDB0hmwfthSV18c2jlYT9k4Zw_-_FkPyQ0wZd3hWJxvk9FysTKPyHX1fVm1i8dOLfwEKYRssQ priority: 102 providerName: ProQuest |
| Title | Gpnmb Is a Potential Marker for the Visceral Pathology in Niemann-Pick Type C Disease |
| URI | https://www.ncbi.nlm.nih.gov/pubmed/26771826 https://www.proquest.com/docview/1757262428 https://www.proquest.com/docview/1760876547 https://pubmed.ncbi.nlm.nih.gov/PMC4714856 https://doaj.org/article/c874fe3fd2384705a0393d03f401a3eb http://dx.doi.org/10.1371/journal.pone.0147208 |
| Volume | 11 |
| WOSCitedRecordID | wos000368628300072&url=https%3A%2F%2Fcvtisr.summon.serialssolutions.com%2F%23%21%2Fsearch%3Fho%3Df%26include.ft.matches%3Dt%26l%3Dnull%26q%3D |
| hasFullText | 1 |
| inHoldings | 1 |
| isFullTextHit | |
| isPrint | |
| journalDatabaseRights | – providerCode: PRVAON databaseName: DOAJ Directory of Open Access Journals customDbUrl: eissn: 1932-6203 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0053866 issn: 1932-6203 databaseCode: DOA dateStart: 20060101 isFulltext: true titleUrlDefault: https://www.doaj.org/ providerName: Directory of Open Access Journals – providerCode: PRVHPJ databaseName: ROAD: Directory of Open Access Scholarly Resources (ISSN International Center) customDbUrl: eissn: 1932-6203 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0053866 issn: 1932-6203 databaseCode: M~E dateStart: 20060101 isFulltext: true titleUrlDefault: https://road.issn.org providerName: ISSN International Centre – providerCode: PRVPQU databaseName: Advanced Technologies & Aerospace Database customDbUrl: eissn: 1932-6203 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0053866 issn: 1932-6203 databaseCode: P5Z dateStart: 20061201 isFulltext: true titleUrlDefault: https://search.proquest.com/hightechjournals providerName: ProQuest – providerCode: PRVPQU databaseName: Agricultural Science Database customDbUrl: eissn: 1932-6203 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0053866 issn: 1932-6203 databaseCode: M0K dateStart: 20061201 isFulltext: true titleUrlDefault: https://search.proquest.com/agriculturejournals providerName: ProQuest – providerCode: PRVPQU databaseName: Biological Science Database (ProQuest) customDbUrl: eissn: 1932-6203 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0053866 issn: 1932-6203 databaseCode: M7P dateStart: 20061201 isFulltext: true titleUrlDefault: http://search.proquest.com/biologicalscijournals providerName: ProQuest – providerCode: PRVPQU databaseName: Engineering Database customDbUrl: eissn: 1932-6203 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0053866 issn: 1932-6203 databaseCode: M7S dateStart: 20061201 isFulltext: true titleUrlDefault: http://search.proquest.com providerName: ProQuest – providerCode: PRVPQU databaseName: Environmental Science Database customDbUrl: eissn: 1932-6203 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0053866 issn: 1932-6203 databaseCode: PATMY dateStart: 20061201 isFulltext: true titleUrlDefault: http://search.proquest.com/environmentalscience providerName: ProQuest – providerCode: PRVPQU databaseName: Health & Medical Collection (ProQuest) customDbUrl: eissn: 1932-6203 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0053866 issn: 1932-6203 databaseCode: 7X7 dateStart: 20061201 isFulltext: true titleUrlDefault: https://search.proquest.com/healthcomplete providerName: ProQuest – providerCode: PRVPQU databaseName: Materials Science Database customDbUrl: eissn: 1932-6203 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0053866 issn: 1932-6203 databaseCode: KB. dateStart: 20061201 isFulltext: true titleUrlDefault: http://search.proquest.com/materialsscijournals providerName: ProQuest – providerCode: PRVPQU databaseName: Nursing & Allied Health Database customDbUrl: eissn: 1932-6203 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0053866 issn: 1932-6203 databaseCode: 7RV dateStart: 20061201 isFulltext: true titleUrlDefault: https://search.proquest.com/nahs providerName: ProQuest – providerCode: PRVPQU databaseName: ProQuest Central Database Suite (ProQuest) customDbUrl: eissn: 1932-6203 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0053866 issn: 1932-6203 databaseCode: BENPR dateStart: 20061201 isFulltext: true titleUrlDefault: https://www.proquest.com/central providerName: ProQuest – providerCode: PRVPQU databaseName: Public Health Database (ProQuest) customDbUrl: eissn: 1932-6203 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0053866 issn: 1932-6203 databaseCode: 8C1 dateStart: 20061201 isFulltext: true titleUrlDefault: https://search.proquest.com/publichealth providerName: ProQuest – providerCode: PRVPQU databaseName: Publicly Available Content Database customDbUrl: eissn: 1932-6203 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0053866 issn: 1932-6203 databaseCode: PIMPY dateStart: 20061201 isFulltext: true titleUrlDefault: http://search.proquest.com/publiccontent providerName: ProQuest – providerCode: PRVATS databaseName: Public Library of Science (PLoS) Journals Open Access customDbUrl: eissn: 1932-6203 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0053866 issn: 1932-6203 databaseCode: FPL dateStart: 20060101 isFulltext: true titleUrlDefault: http://www.plos.org/publications/ providerName: Public Library of Science |
| link | http://cvtisr.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwlV3db9MwELeg44EXYHytUIpBSMBDSj7t5HEtK0xjJepYVXiJHMeGiC2tmhaJ_547Jw1k2gS8nNT6bCVn-_y72PczIS8iAMlpGjgW01lk-YIJK_K1tAA9My61kwohzWUTfDIJ5_Mo_h0oXtjB97jzprbpYLko1AAAPXcxt3fH9RjDqxrG8Yet54W5y1idHndVzdbyY1j6G1_cWZ4tysuA5sXzkn8sQOPb__vod8itGmrS_Wps7JJrqrhLduvJXNJXNeP063vk9N2yOE_pYUkFjRdrPEAEFTGNR60ooFoKKJHO8lKiPo3F2rjMnzQv6CRX56IorDiX3ykGtXRE31abPvfJ6fjg0-i9Vd-3YEmIGtYWU7BgphARMt_ObM6yzHV0FmQ84iKUrpulQuso8qNMppn2POW5kYoYd5SXSs6F94B0CnjVPUI1NMShr6Ey8yP4ISEw0k7EkcvHdrIu8bbdkMiajBzvxDhLzA4bh6CkMlKCtktq23WJ1dRaVmQcf9EfYg83ukilbf6ATkrqmZnIkPtaeToD8OJzOxCYrZzZnobIU3gq7ZKnOD6SKi-1cQjJvu8bsh-bd8lzo4F0GgWe1_kqNmWZHH6c_YPSybSl9LJW0gswhxR1jgS8E9J0tTR7LU1wCrJVvIejeWuVMgGUyF3MBQKj9LYj_PLiZ00xNopn8Aq12KAOQ_7CwIfWH1YTorGsyzjHSLVLeGuqtEzfLinyb4bNHNCRHwbs0dVP_JjcBJhqPnw5QY901quNekJuyB_rvFz1yXU-naGccyNDkOHI6ZOd4cEknvbNV5a-cRQgj4YDkMf2EUoeG3kCMg6-QI348Dj-_At6oGdg |
| linkProvider | Public Library of Science |
| linkToHtml | http://cvtisr.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMw1V1Lb9NAEF6VgAQXoLwaKHRBIODgNvbaXu8BoZJSGrWEqLRVb2a9j2LR2iFOQP1T_EZmbMfUqAIuPXCMd3YVj-dpz3xDyFMBQXKSBK4TWi0cX4bSEb5VDkTPIVfWTaRU5bAJPhxGh4ditEB-zHthsKxybhNLQ61zhe_I18DNcQ-bGaLX468OTo3Cr6vzERqVWGyb0--QshWvBhvwfJ953ubbvf6WU08VcBTExlMnNOAWEsh7Qr-nIYvX2nOtDjQXXEbK83QirRXCF1ol2jJmmCeMCLlrWKI4lwzOvUQu-8xjqEVRvykpAdsRhnV7HuPuWi0Nq-M8M6uQinAPh1iecX_llIDGF3TGx3lxXqD7e73mGQe4eeN_Y91Ncr0Otel6pRuLZMFkt8hibcwK-qJG3H55m-y_G2cnCR0UVNJRPsUCKtiIbUxmQiGqpxAl04O0UEhPR3JauoxTmmZ0mJoTmWXOKFVfKCb1tE83qo9ed8j-hdzeXdLJ4FkvEWrhIA6yDptDX8APBYmhdQVHLKOeq7uEzcUgVjUYO84EOY7LL4wckrKKSTEKT1wLT5c4za5xBUbyF_o3KGENLUKJlxfyyVFcW6ZYRdy3hlkNwZvPe4HEbm3dYxYyb8lM0iUrKJ9x1ZfbGMR43fdLsKMe75InJQXCiWRYr3QkZ0URDz4c_APRx90W0fOayObADiXrHhG4J4Qpa1EutyjBKKrW8hJq05wrRfxLB2DnXEvOX37cLOOhWIOYmXyGNCHiNwY-nH6vUsiGs17IOWbqXcJbqtpifXslSz-XaO4QHfpREN7_899aIVe39t7vxDuD4fYDcg3C9vJFoBssk850MjMPyRX1bZoWk0elSaLk00Ur8k_xo8v7 |
| linkToPdf | http://cvtisr.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMw1V1Lb9NAEF6VgBAXoLwaKHRBIODgxs_d-IBQaQhERcGitOrNrPdRLFo7xAmof41fx4xf1KgCLj1wjHd2FY_nac98Q8jjEILkJAkcixkVWr5gwgp9Iy2InhmXxkmEkOWwCT6dDg8OwmiF_Gh6YbCssrGJpaFWucR35ANwc9zFZobhwNRlEdFo_HL21cIJUviltRmnUYnIjj75Dulb8WIygmf9xHXHrz9uv7XqCQOWhDh5YTENLiKBHIj5toKMXinXMSpQPORiKF1XJcKYMPRDJRNlPE97bqhDxh3tJZJz4cG5F8hF7jPbLcsGo8YLgB1hrG7V87gzqCVjc5ZnehPSEu7iQMtTrrCcGND6hd7sKC_OCnp_r9085QzH1_5nNl4nV-sQnG5VOrNKVnR2g6zWRq6gz2ok7uc3yd6bWXac0ElBBY3yBRZWwUZsb9JzCtE-heiZ7qeFRHoaiUXpSk5omtFpqo9FlllRKr9QTPbpNh1VH8Nukb1zub3bpJfBc18j1MBBHHQANjM_hB8SEkbjhBwxjmxH9YnXiEQsa5B2nBVyFJdfHjkkaxWTYhSkuBakPrHaXbMKpOQv9K9Q2lpahBgvL-Tzw7i2WLEcct9ozygI6nxuBwK7uJXtGcjIhaeTPtlAWY2rft3WUMZbvl-CINm8Tx6VFAgzkqGkHYplUcST9_v_QLT7oUP0tCYyObBDirp3BO4J4cs6lOsdSjCWsrO8hprVcKWIf-kD7Gw05uzlh-0yHoq1iZnOl0jDENcx8OH0O5Vytpx1GeeYwfcJ76hth_XdlSz9XKK8Q9ToDwN2989_a4NcBv2N302mO_fIFYjmy_eDTrBOeov5Ut8nl-S3RVrMH5TWiZJP563HPwHh2NUH |
| openUrl | ctx_ver=Z39.88-2004&ctx_enc=info%3Aofi%2Fenc%3AUTF-8&rfr_id=info%3Asid%2Fsummon.serialssolutions.com&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Gpnmb+Is+a+Potential+Marker+for+the+Visceral+Pathology+in+Niemann-Pick+Type+C+Disease&rft.jtitle=PloS+one&rft.au=Marques%2C+Andr%C3%A9+R+A&rft.au=Gabriel%2C+Tanit+L&rft.au=Aten%2C+Jan&rft.au=van+Roomen%2C+Cindy+P+A+A&rft.date=2016-01-15&rft.eissn=1932-6203&rft.volume=11&rft.issue=1&rft.spage=e0147208&rft.epage=e0147208&rft_id=info:doi/10.1371%2Fjournal.pone.0147208&rft.externalDBID=NO_FULL_TEXT |
| thumbnail_l | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/lc.gif&issn=1932-6203&client=summon |
| thumbnail_m | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/mc.gif&issn=1932-6203&client=summon |
| thumbnail_s | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/sc.gif&issn=1932-6203&client=summon |