{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T07:49:31Z","timestamp":1782978571840,"version":"3.54.5"},"reference-count":37,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2022,6,29]],"date-time":"2022-06-29T00:00:00Z","timestamp":1656460800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Neuroscience Research"],"published-print":{"date-parts":[[2022,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    The most frequent genetic cause of frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS) is the hexanucleotide repeat expansion in\n                    <jats:italic>C9orf72<\/jats:italic>\n                    . An important neuropathological hallmark associated with this mutation is the accumulation of the phosphorylated form of TAR (trans\u2010activation response element) DNA\u2010binding protein 43 (pTDP\u201043). Glia plays a crucial role in the neurodegeneration observed in\n                    <jats:italic>C9orf72<\/jats:italic>\n                    \u2010associated disorders. However, less is known about the role of oligodendrocytes (OLs). Here, we applied digital neuropathological methods to compare the expression pattern of glial cells in the frontal cortex (FrCx) of human post\u2010mortem samples from patients with C9\u2010FTLD and C9\u2010FTLD\/ALS, sporadic FTLD (sFTLD), and healthy controls (HCs). We also compared MBP levels in CSF from an independent clinical FTD cohort. We observed an increase in GFAP, and Iba1 immunoreactivity in C9 and sFTLD compared to controls in the gray matter (GM) of the FrCx. We observed a decrease in MBP immunoreactivity in the GM and white matter (WM) of the FrCx of C9, compared to HC and sFTLD. There was a negative correlation between MBP and pTDP\u201043 in C9 in the WM of the FrCx. We observed an increase in CSF MBP concentrations in C9 and sFTLD compared to HC. In conclusion, the C9 expansion is associated with myelin loss in the frontal cortex. This loss of MBP may be a result of oligodendroglial dysfunction due to the expansion or the presence of pTDP\u201043 in OLs. Understanding these biological processes will help to identify specific pathways associated with the\n                    <jats:italic>C9orf72<\/jats:italic>\n                    expansion.\n                  <\/jats:p>","DOI":"10.1002\/jnr.25100","type":"journal-article","created":{"date-parts":[[2022,6,29]],"date-time":"2022-06-29T07:28:33Z","timestamp":1656487713000},"page":"1862-1875","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Myelin loss in\n                    <i>C9orf72<\/i>\n                    hexanucleotide expansion carriers"],"prefix":"10.1002","volume":"100","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5366-7832","authenticated-orcid":false,"given":"S\u00f2nia","family":"Sirisi","sequence":"first","affiliation":[{"name":"Memory Unit, Neurology Department and Sant Pau Biomedical Research Institute, Hospital de la Santa Creu i Sant Pau Universitat Aut\u00f2noma de Barcelona  Barcelona Spain"},{"name":"Network Center for Biomedical Research in Neurodegenerative Diseases (CIBERNED)  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Querol\u2010Vilaseca","sequence":"additional","affiliation":[{"name":"Memory Unit, Neurology Department and Sant Pau Biomedical Research Institute, Hospital de la Santa Creu i Sant Pau Universitat Aut\u00f2noma de Barcelona  Barcelona Spain"},{"name":"Network Center for Biomedical Research in Neurodegenerative Diseases (CIBERNED)  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oriol","family":"Dols\u2010Icardo","sequence":"additional","affiliation":[{"name":"Memory Unit, Neurology Department and Sant Pau Biomedical Research Institute, Hospital de la Santa Creu i Sant Pau Universitat Aut\u00f2noma de Barcelona  Barcelona Spain"},{"name":"Network Center for Biomedical Research in Neurodegenerative Diseases (CIBERNED)  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jordi","family":"Pegueroles","sequence":"additional","affiliation":[{"name":"Memory Unit, Neurology Department and Sant Pau Biomedical Research Institute, Hospital de la Santa Creu i Sant Pau Universitat Aut\u00f2noma de Barcelona  Barcelona Spain"},{"name":"Network Center for Biomedical Research in Neurodegenerative Diseases (CIBERNED)  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victor","family":"Montal","sequence":"additional","affiliation":[{"name":"Memory Unit, Neurology Department and Sant Pau Biomedical Research Institute, Hospital de la Santa Creu i Sant Pau Universitat Aut\u00f2noma de Barcelona  Barcelona Spain"},{"name":"Network Center for Biomedical Research in Neurodegenerative Diseases (CIBERNED)  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laia","family":"Mu\u00f1oz","sequence":"additional","affiliation":[{"name":"Memory Unit, Neurology Department and Sant Pau Biomedical Research Institute, Hospital de la Santa Creu i Sant Pau Universitat Aut\u00f2noma de Barcelona  Barcelona Spain"},{"name":"Network Center for Biomedical Research in Neurodegenerative Diseases (CIBERNED)  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Soraya","family":"Torres","sequence":"additional","affiliation":[{"name":"Memory Unit, Neurology Department and Sant Pau Biomedical Research Institute, Hospital de la Santa Creu i Sant Pau Universitat Aut\u00f2noma de Barcelona  Barcelona Spain"},{"name":"Network Center for Biomedical Research in Neurodegenerative Diseases (CIBERNED)  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paula","family":"Ferrer\u2010Ravent\u00f3s","sequence":"additional","affiliation":[{"name":"Memory Unit, Neurology Department and Sant Pau Biomedical Research Institute, Hospital de la Santa Creu i Sant Pau Universitat Aut\u00f2noma de Barcelona  Barcelona Spain"},{"name":"Network Center for Biomedical Research in Neurodegenerative Diseases (CIBERNED)  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Florencia","family":"Iulita","sequence":"additional","affiliation":[{"name":"Memory Unit, Neurology Department and Sant Pau Biomedical Research Institute, Hospital de la Santa Creu i Sant Pau Universitat Aut\u00f2noma de Barcelona  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c9rika","family":"S\u00e1nchez\u2010Aced","sequence":"additional","affiliation":[{"name":"Memory Unit, Neurology Department and Sant Pau Biomedical Research Institute, Hospital de la Santa Creu i Sant Pau Universitat Aut\u00f2noma de Barcelona  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rafael","family":"Blesa","sequence":"additional","affiliation":[{"name":"Memory Unit, Neurology Department and Sant Pau Biomedical Research Institute, Hospital de la Santa Creu i Sant Pau Universitat Aut\u00f2noma de Barcelona  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ignacio","family":"Ill\u00e1n\u2010Gala","sequence":"additional","affiliation":[{"name":"Memory Unit, Neurology Department and Sant Pau Biomedical Research Institute, Hospital de la Santa Creu i Sant Pau Universitat Aut\u00f2noma de Barcelona  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Molina\u2010Porcel","sequence":"additional","affiliation":[{"name":"Neurological Tissue Bank of the Biobanc\u2010Hospital Clinic\u2010IDIBAPS Institut d'Investigacions Biom\u00e8diques August Pi i Sunyer  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sergi","family":"Borrego\u2010Ecija","sequence":"additional","affiliation":[{"name":"Alzheimer's Disease and Other Cognitive Disorders Unit, Service of Neurology, Hospital Cl\u00ednic Institut d'Investigaci\u00f3 Biom\u00e8dica August Pi i Sunyer, University of Barcelona  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raquel","family":"S\u00e1nchez\u2010Valle","sequence":"additional","affiliation":[{"name":"Neurological Tissue Bank of the Biobanc\u2010Hospital Clinic\u2010IDIBAPS Institut d'Investigacions Biom\u00e8diques August Pi i Sunyer  Barcelona Spain"},{"name":"Alzheimer's Disease and Other Cognitive Disorders Unit, Service of Neurology, Hospital Cl\u00ednic Institut d'Investigaci\u00f3 Biom\u00e8dica August Pi i Sunyer, University of Barcelona  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jordi","family":"Clarimon","sequence":"additional","affiliation":[{"name":"Memory Unit, Neurology Department and Sant Pau Biomedical Research Institute, Hospital de la Santa Creu i Sant Pau Universitat Aut\u00f2noma de Barcelona  Barcelona 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