{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T04:59:24Z","timestamp":1792213164733,"version":"4.4.0"},"reference-count":59,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2023,2,15]],"date-time":"2023-02-15T00:00:00Z","timestamp":1676419200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000780","name":"European Commission","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100000780","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100010438","name":"Francis Crick Institute","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100010438","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100000289","name":"Cancer Research UK","doi-asserted-by":"crossref","award":["FC010110; 215593\/Z\/19\/Z"],"award-info":[{"award-number":["FC010110; 215593\/Z\/19\/Z"]}],"id":[{"id":"10.13039\/501100000289","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100000265","name":"UK Medical Research Council","doi-asserted-by":"crossref","award":["FC010110"],"award-info":[{"award-number":["FC010110"]}],"id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"crossref","award":["FC010110"],"award-info":[{"award-number":["FC010110"]}],"id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Okinawa Institute of Science & Technology Graduate University"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,6,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Valosin-containing protein (VCP) is a hexameric ATPase associated with diverse cellular activities. Genetic mutations in VCP are associated with several forms of muscular and neuronal degeneration, including amyotrophic lateral sclerosis (ALS). Moreover, VCP mediates UV-induced proteolysis of RNA polymerase II (RNAPII), but little is known about the effects of VCP mutations on the transcriptional machinery. Here, we used silica particle-assisted chromatin enrichment and mass spectrometry to study proteins co-localized with RNAPII in precursor neurons differentiated from VCP-mutant or control induced pluripotent stem cells. Remarkably, we observed diminished RNAPII binding of proteins involved in transcription elongation and mRNA splicing in mutant cells. One of these is SART3, a recycling factor of the splicing machinery, whose knockdown leads to perturbed intron retention in several ALS-associated genes. Additional reduced proteins are RBM45, EIF5A and RNF220, mutations in which are associated with various neurodegenerative disorders and are linked to TDP-43 aggregation. Conversely, we observed increased RNAPII binding of heat shock proteins such as HSPB1. Together, these findings shed light on how transcription and splicing machinery are impaired by VCP mutations, which might contribute to aberrant alternative splicing and proteinopathy in neurodegeneration.<\/jats:p>","DOI":"10.1093\/brain\/awad046","type":"journal-article","created":{"date-parts":[[2023,2,13]],"date-time":"2023-02-13T23:40:02Z","timestamp":1676331602000},"page":"2547-2556","source":"Crossref","is-referenced-by-count":11,"title":["RNA polymerase II-associated proteins reveal pathways affected in VCP-related amyotrophic lateral sclerosis"],"prefix":"10.1093","volume":"146","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1317-1162","authenticated-orcid":false,"given":"Mahmoud-Reza","family":"Rafiee","sequence":"first","affiliation":[{"name":"The Francis Crick Institute , London NW1 1AT , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Rohban","sequence":"additional","affiliation":[{"name":"The Francis Crick Institute , London NW1 1AT , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen","family":"Davey","sequence":"additional","affiliation":[{"name":"The Francis Crick Institute , London NW1 1AT , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jernej","family":"Ule","sequence":"additional","affiliation":[{"name":"The Francis Crick Institute , London NW1 1AT , UK"},{"name":"Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology , London, WC1N 3BG , UK"},{"name":"UK Dementia Research Institute Centre, King\u2019s College London , London, SE5 9RX , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicholas M","family":"Luscombe","sequence":"additional","affiliation":[{"name":"The Francis Crick Institute , London NW1 1AT , UK"},{"name":"UCL Genetics Institute, University College London , London, WC1E 6BT , UK"},{"name":"Genomics and Regulatory Systems Unit, Okinawa Institute of Science & Technology Graduate University , Okinawa 904-0495 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,2,15]]},"reference":[{"key":"2023072814571890500_awad046-B1","doi-asserted-by":"crossref","first-page":"39","DOI":"10.3389\/fmolb.2017.00039","article-title":"A mighty \u201cprotein extractor\u201d of the cell: Structure and function of the p97\/CDC48 ATPase","volume":"4","author":"Ye","year":"2017","journal-title":"Front Mol Biosci"},{"key":"2023072814571890500_awad046-B2","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1016\/j.molcel.2017.10.028","article-title":"VCP\/p97-Mediated unfolding as a principle in protein homeostasis and signaling","volume":"69","author":"van den Boom","year":"2018","journal-title":"Mol Cell."},{"key":"2023072814571890500_awad046-B3","doi-asserted-by":"crossref","first-page":"70","DOI":"10.3389\/fnmol.2014.00070","article-title":"The ubiquitin\u2013proteasome system in neurodegenerative diseases: Precipitating factor, 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