{"PubmedArticle":{"MedlineCitation":{"@attributes":{"Status":"MEDLINE","Owner":"NLM","IndexingMethod":"Automated"},"PMID":{"@attributes":{"Version":"1"},"@text":"39673425"},"DateCompleted":{"Year":"2025","Month":"04","Day":"03"},"DateRevised":{"Year":"2025","Month":"05","Day":"18"},"Article":{"@attributes":{"PubModel":"Print"},"Journal":{"ISSN":{"@attributes":{"IssnType":"Electronic"},"@text":"1460-2156"},"JournalIssue":{"@attributes":{"CitedMedium":"Internet"},"Volume":"148","Issue":"4","PubDate":{"Year":"2025","Month":"Apr","Day":"03"}},"Title":"Brain : a journal of neurology","ISOAbbreviation":"Brain"},"ArticleTitle":"The role of dystrophin isoforms and interactors in the brain.","Pagination":{"StartPage":"1081","EndPage":"1098","MedlinePgn":"1081-1098"},"ELocationID":[{"@attributes":{"EIdType":"doi","ValidYN":"Y"},"@text":"10.1093\/brain\/awae384"}],"Abstract":{"AbstractText":["Dystrophin is a protein crucial for maintaining the structural integrity of skeletal muscle. So far, attention has been focused on the role of dystrophin in muscle, in view of the devastating progression of weakness and early death that characterizes Duchenne muscular dystrophy. However, in the last few years, the role of shorter dystrophin isoforms, including development and adult expression-specific mechanisms, has been a greater focus. Within the cerebral landscape, various cell types, such as glia, oligodendrocytes and Purkinje, cerebellar granule and vascular-associated cells express a spectrum of dystrophin isoforms, including Dp427, Dp140, Dp71 and Dp40. The interaction of these isoforms with a multitude of proteins suggests their involvement in neurotransmission, influencing several circuit functions. This review presents the intricate interactions among dystrophin isoforms and diverse protein complexes across different cell types and brain regions, as well as the associated clinical complications. We focus on studies investigating protein interactions with dystrophin in the past 30\u2005years at a biochemical level. In essence, the brain's dystrophin landscape is a thrilling exploration of diversity, challenging preconceptions and opening new avenues for understanding CNS physiology. It also holds potential therapeutic implications for neurological complications involving brain dystrophin deficiency. By revealing the molecular complexities related to dystrophin, this review paves the way for future investigations and therapeutic interventions for this CNS aspect of Duchenne muscular dystrophy."],"CopyrightInformation":"\u00a9 The Author(s) 2024. Published by Oxford University Press on behalf of the Guarantors of Brain."},"AuthorList":{"@attributes":{"CompleteYN":"Y"},"Author":[{"@attributes":{"ValidYN":"Y"},"LastName":"Tetorou","ForeName":"Konstantina","Initials":"K","AffiliationInfo":[{"Affiliation":"Developmental Neurosciences Department, Dubowitz Neuromuscular Centre, University College London, Great Ormond Street Institute of Child Health, London WC1N 1EH, UK."},{"Affiliation":"Developmental Neurosciences Department, National Institute for Health Research Great Ormond Street Hospital Biomedical Research Centre, London WC1N 1EH, UK."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Aghaeipour","ForeName":"Artadokht","Initials":"A","AffiliationInfo":[{"Affiliation":"Developmental Neurosciences Department, Dubowitz Neuromuscular Centre, University College London, Great Ormond Street Institute of Child Health, London WC1N 1EH, UK."},{"Affiliation":"Developmental Neurosciences Department, National Institute for Health Research Great Ormond Street Hospital Biomedical Research Centre, London WC1N 1EH, UK."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Singh","ForeName":"Simran","Initials":"S","AffiliationInfo":[{"Affiliation":"Developmental Neurosciences Department, Dubowitz Neuromuscular Centre, University College London, Great Ormond Street Institute of Child Health, London WC1N 1EH, UK."},{"Affiliation":"Developmental Neurosciences Department, National Institute for Health Research Great Ormond Street Hospital Biomedical Research Centre, London WC1N 1EH, UK."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Morgan","ForeName":"Jennifer E","Initials":"JE","AffiliationInfo":[{"Affiliation":"Developmental Neurosciences Department, Dubowitz Neuromuscular Centre, University College London, Great Ormond Street Institute of Child Health, London WC1N 1EH, UK."},{"Affiliation":"Developmental Neurosciences Department, National Institute for Health Research Great Ormond Street Hospital Biomedical Research Centre, London WC1N 1EH, UK."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Muntoni","ForeName":"Francesco","Initials":"F","AffiliationInfo":[{"Affiliation":"Developmental Neurosciences Department, Dubowitz Neuromuscular Centre, University College London, Great Ormond Street Institute of Child Health, London WC1N 1EH, UK."},{"Affiliation":"Developmental Neurosciences Department, National Institute for Health Research Great Ormond Street Hospital Biomedical Research Centre, London WC1N 1EH, UK."}]}]},"Language":["eng"],"GrantList":{"@attributes":{"CompleteYN":"Y"},"Grant":[{"GrantID":"#847826","Agency":"European Commission","Country":""}]},"PublicationTypeList":{"PublicationType":[{"@attributes":{"UI":"D016428"},"@text":"Journal Article"},{"@attributes":{"UI":"D016454"},"@text":"Review"}]}},"MedlineJournalInfo":{"Country":"England","MedlineTA":"Brain","NlmUniqueID":"0372537","ISSNLinking":"0006-8950"},"ChemicalList":{"Chemical":[{"RegistryNumber":"0","NameOfSubstance":{"@attributes":{"UI":"D016189"},"@text":"Dystrophin"}},{"RegistryNumber":"0","NameOfSubstance":{"@attributes":{"UI":"D020033"},"@text":"Protein Isoforms"}}]},"CitationSubset":["IM"],"MeshHeadingList":{"MeshHeading":[{"DescriptorName":{"@attributes":{"UI":"D006801","MajorTopicYN":"N"},"@text":"Humans"}},{"DescriptorName":{"@attributes":{"UI":"D016189","MajorTopicYN":"Y"},"@text":"Dystrophin"},"QualifierName":[{"@attributes":{"UI":"Q000378","MajorTopicYN":"N"},"@text":"metabolism"}]},{"DescriptorName":{"@attributes":{"UI":"D020033","MajorTopicYN":"N"},"@text":"Protein Isoforms"},"QualifierName":[{"@attributes":{"UI":"Q000378","MajorTopicYN":"N"},"@text":"metabolism"}]},{"DescriptorName":{"@attributes":{"UI":"D000818","MajorTopicYN":"N"},"@text":"Animals"}},{"DescriptorName":{"@attributes":{"UI":"D001921","MajorTopicYN":"Y"},"@text":"Brain"},"QualifierName":[{"@attributes":{"UI":"Q000378","MajorTopicYN":"N"},"@text":"metabolism"}]},{"DescriptorName":{"@attributes":{"UI":"D020388","MajorTopicYN":"N"},"@text":"Muscular Dystrophy, Duchenne"},"QualifierName":[{"@attributes":{"UI":"Q000378","MajorTopicYN":"N"},"@text":"metabolism"}]}]},"KeywordList":[{"@attributes":{"Owner":"NOTNLM"},"Keyword":[{"@attributes":{"MajorTopicYN":"N"},"@text":"brain"},{"@attributes":{"MajorTopicYN":"N"},"@text":"brain comorbidities"},{"@attributes":{"MajorTopicYN":"N"},"@text":"duchenne muscular dystrophy"},{"@attributes":{"MajorTopicYN":"N"},"@text":"dystrophin"},{"@attributes":{"MajorTopicYN":"N"},"@text":"protein interactions"}]}],"CoiStatement":"F.M. has received compensation for participation to advisory boards from Sarepta, Dyne Therapeutics, Edgewise, Roche, PTC Therapeutics, Solid Bioscience. His institute receives funding from Sarepta for investigator-initiated studies, and from Sarepta, Roche, Pfizer for participation in clinical trials. The other authors report no competing interests."},"PubmedData":{"History":{"PubMedPubDate":[{"@attributes":{"PubStatus":"received"},"Year":"2024","Month":"4","Day":"24"},{"@attributes":{"PubStatus":"revised"},"Year":"2024","Month":"9","Day":"23"},{"@attributes":{"PubStatus":"accepted"},"Year":"2024","Month":"10","Day":"26"},{"@attributes":{"PubStatus":"medline"},"Year":"2025","Month":"4","Day":"3","Hour":"18","Minute":"25"},{"@attributes":{"PubStatus":"pubmed"},"Year":"2024","Month":"12","Day":"14","Hour":"20","Minute":"6"},{"@attributes":{"PubStatus":"entrez"},"Year":"2024","Month":"12","Day":"14","Hour":"6","Minute":"43"},{"@attributes":{"PubStatus":"pmc-release"},"Year":"2024","Month":"12","Day":"2"}]},"PublicationStatus":"ppublish","ArticleIdList":{"ArticleId":[{"@attributes":{"IdType":"pubmed"},"@text":"39673425"},{"@attributes":{"IdType":"pmc"},"@text":"PMC11967788"},{"@attributes":{"IdType":"doi"},"@text":"10.1093\/brain\/awae384"},{"@attributes":{"IdType":"pii"},"@text":"7914977"}]},"ReferenceList":[{"Reference":[{"Citation":"Den Dunnen \u00a0JT, Grootscholten \u00a0RM, Bakker \u00a0E, et al. \u00a0Topography of the Duchenne Muscular Dystrophy (DMD) gene: FIGE and CDNA analysis of 194 cases reveals 115 deletions and 13 duplications. 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