{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T13:51:16Z","timestamp":1781790676241,"version":"3.54.5"},"reference-count":82,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2026,6,1]],"date-time":"2026-06-01T00:00:00Z","timestamp":1780272000000},"content-version":"vor","delay-in-days":151,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005309","name":"Alzheimer\u2019s Society","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005309","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002283","name":"Alzheimer\u2019s Research UK","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002283","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100003607","name":"Association of Frontotemporal Dementia","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100003607","id-type":"DOI","asserted-by":"publisher"}]},{"name":"BRAIN non-clinical fellowship"},{"name":"My Name\u20195 Doddie Foundation"},{"name":"Race Against Dementia Ignition Fund"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,5,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Frontotemporal dementia is a neurodegenerative disorder with a strong heritable component. Frontotemporal lobar degeneration refers to the pathological changes seen in frontotemporal dementia, characterized by atrophy of the frontal and temporal lobes and the presence of abnormal protein inclusions. In the case of frontotemporal lobar degeneration with hyperphosphorylated TDP-43 positive inclusions (FTLD-TDP), five pathological subtypes (A, B, C, D and E) are observed based on the types and distribution of inclusions found in the brain. In all subtypes, there tends to be a large variability in the number of pathological inclusions observed between cases, with limited correlation to clinical manifestations.<\/jats:p>\n                  <jats:p>TDP-43 is an RNA-binding protein belonging to the heterogeneous nuclear ribonucleoprotein (hnRNP) family, which along with other hnRNPs, modulates multiple aspects of RNA processing. HnRNPs other than TDP-43 have been implicated in several neurological diseases, including Amyotrophic Lateral Sclerosis, FTLD-TDP, frontotemporal lobar degeneration with fused in sarcoma (FTLD-FUS) and Alzheimer\u2019s disease. Multiple hnRNPs have been found in pathological inclusions in specific subtypes of FTLD-TDP, suggesting potential roles in the disease process. The role of the hnRNP network in frontotemporal lobar degeneration disease pathogenesis, however, has not yet been investigated. This study aimed to comprehensively evaluate the presence and expression of hnRNP proteins in two pathological subtypes of sporadic FTLD-TDP (A and C) as well as the genetic form FTLD-TDP A C9orf72 using immunohistochemistry and gene expression analysis by single-nuclei RNA-sequencing.<\/jats:p>\n                  <jats:p>We found that there was great variability in the frequency of TDP-43 pathology across and within FTLD-TDP pathological subtypes. Our findings suggest that distinct global transcriptomic profiles may underlie the different pathological subtypes of FTLD-TDP. The most prominent transcriptomic changes were observed in oligodendrocytes and astrocytes, involving multiple hnRNPs across frontotemporal lobar degeneration subtypes compared to controls. Transcriptomic co-expression analysis further revealed that glial clusters were more strongly associated with RNA-processing dysfunction and contributed to disease classification. Together, these findings highlight the involvement of the hnRNP network and glial-specific RNA-processing alterations in FTLD-TDP pathophysiology, offering new insight into the molecular distinctions between pathological subtypes and potential targets for future investigation.<\/jats:p>","DOI":"10.1093\/braincomms\/fcag197","type":"journal-article","created":{"date-parts":[[2026,5,30]],"date-time":"2026-05-30T11:43:21Z","timestamp":1780141401000},"source":"Crossref","is-referenced-by-count":0,"title":["Transcriptomic and pathological analysis of the hnRNP network reveals glial involvement in frontotemporal lobar degeneration pathological subtypes"],"prefix":"10.1093","volume":"8","author":[{"given":"Ariana","family":"Gatt","sequence":"first","affiliation":[{"name":"Department of Neurodegenerative Diseases, UCL Queen Square Institute of Neurology, University College London , London WC1N 1PJ ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yazead","family":"Buhidma","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Diseases, UCL Queen Square Institute of Neurology, University College London , London WC1N 1PJ ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine","family":"Fodder","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Diseases, UCL Queen Square Institute of Neurology, University College London , London WC1N 1PJ ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jack","family":"Humphrey","sequence":"additional","affiliation":[{"name":"Icahn School of Medicine at Mount Sinai Nash Family Department of Neuroscience and Friedman Brain Institute, , NewYork City, NY 10029 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandrine C","family":"Foti","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Diseases, UCL Queen Square Institute of Neurology, University College London , London WC1N 1PJ ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara","family":"Frias","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Diseases, UCL Queen Square Institute of Neurology, University College London , London WC1N 1PJ ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bridget C","family":"Benson","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield , Sheffield S10 2HQ ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Priya","family":"Gami-Patel","sequence":"additional","affiliation":[{"name":"Department of Pathology, Amsterdam Neuroscience, Amsterdam University Medical Centers , Amsterdam ,","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauren M","family":"Gittings","sequence":"additional","affiliation":[{"name":"Department of Translational Neuroscience, Barrow Neurological Institute , Phoenix AZ 85013 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina E","family":"Toomey","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Diseases, UCL Queen Square Institute of Neurology, University College London , London WC1N 1PJ ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7389-0348","authenticated-orcid":false,"given":"Tammaryn","family":"Lashley","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Diseases, UCL Queen Square Institute of Neurology, University College London , London WC1N 1PJ 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