{"PubmedArticle":{"MedlineCitation":{"@attributes":{"Status":"MEDLINE","Owner":"NLM","IndexingMethod":"Manual"},"PMID":{"@attributes":{"Version":"1"},"@text":"35098363"},"DateCompleted":{"Year":"2022","Month":"02","Day":"07"},"DateRevised":{"Year":"2024","Month":"05","Day":"16"},"Article":{"@attributes":{"PubModel":"Electronic"},"Journal":{"ISSN":{"@attributes":{"IssnType":"Electronic"},"@text":"1420-9071"},"JournalIssue":{"@attributes":{"CitedMedium":"Internet"},"Volume":"79","Issue":"2","PubDate":{"Year":"2022","Month":"Jan","Day":"30"}},"Title":"Cellular and molecular life sciences : CMLS","ISOAbbreviation":"Cell Mol Life Sci"},"ArticleTitle":"Generation of dystrophin short product-specific tag-insertion mouse: distinct Dp71 glycoprotein complexes at inhibitory postsynapse and glia limitans.","Pagination":{"StartPage":"109","MedlinePgn":"109"},"ELocationID":[{"@attributes":{"EIdType":"pii","ValidYN":"Y"},"@text":"109"},{"@attributes":{"EIdType":"doi","ValidYN":"Y"},"@text":"10.1007\/s00018-022-04151-2"}],"Abstract":{"AbstractText":["Duchenne muscular dystrophy (DMD), the most severe form of dystrophinopathies, is a fatal X-linked recessive neuromuscular disorder characterized by progressive muscle degeneration and various extents of intellectual disabilities. Physiological and pathological roles of the responsible gene, dystrophin, in the brain remain elusive due to the presence of multiple dystrophin products, mainly full-length dystrophin, Dp427, and the short product, Dp71. In this study, we generated a Dp71-specific hemagglutinin (HA) peptide tag-insertion mice to enable specific detection of intrinsic Dp71 expression by anti-HA-tag antibodies. Immunohistochemical detections in the transgenic mice demonstrated Dp71 expression not only at the blood-brain barrier, where astrocytic endfeet surround the microvessels, but also at the inhibitory postsynapse of hippocampal dentate granule neurons. Interestingly, hippocampal cornu ammonis (CA)1 pyramidal neurons were negative for Dp71, although Dp427 detected by anti-dystrophin antibody was clearly present at the inhibitory postsynapse, suggesting cell-type dependent dystrophin expressions. Precise examination using the primary hippocampal culture validated exclusive localization of Dp71 at the inhibitory postsynaptic compartment but not at the excitatory synapse in neurons. We further performed interactome analysis and found that Dp71 formed distinct molecular complexes, i.e. synapse-associated Dp71 interacted with dystroglycan (Dg) and dystrobrevin\u03b2 (Dtnb), whereas glia-associated Dp71 did with Dg and dystrobrevin\u03b1 (Dtna). Thus, our data indicate that Dp71 and its binding partners are relevant to the inhibitory postsynaptic function of hippocampal granule neurons and the novel Dp71-transgenic mouse provides a valuable tool to understand precise physiological expressions and functions of Dp71 and its interaction proteins in vivo and in vitro."],"CopyrightInformation":"\u00a9 2022. The Author(s), under exclusive licence to Springer Nature Switzerland AG."},"AuthorList":{"@attributes":{"CompleteYN":"Y"},"Author":[{"@attributes":{"ValidYN":"Y"},"LastName":"Fujimoto","ForeName":"Takahiro","Initials":"T","Identifier":[{"@attributes":{"Source":"ORCID"},"@text":"0000-0002-9975-7008"}],"AffiliationInfo":[{"Affiliation":"Department of Pathology and Applied Neurobiology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, 465 Kajii-cho, Kawaramachi Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Yaoi","ForeName":"Takeshi","Initials":"T","AffiliationInfo":[{"Affiliation":"Department of Pathology and Applied Neurobiology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, 465 Kajii-cho, Kawaramachi Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Nakano","ForeName":"Kenta","Initials":"K","AffiliationInfo":[{"Affiliation":"Department of Laboratory Animal Medicine, Research Institute, National Center for Global Health and Medicine (NCGM), Tokyo, 162-8655, Japan."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Arai","ForeName":"Tetsuya","Initials":"T","AffiliationInfo":[{"Affiliation":"Department of Laboratory Animal Medicine, Research Institute, National Center for Global Health and Medicine (NCGM), Tokyo, 162-8655, Japan."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Okamura","ForeName":"Tadashi","Initials":"T","AffiliationInfo":[{"Affiliation":"Department of Laboratory Animal Medicine, Research Institute, National Center for Global Health and Medicine (NCGM), Tokyo, 162-8655, Japan."}]},{"@attributes":{"ValidYN":"Y"},"LastName":"Itoh","ForeName":"Kyoko","Initials":"K","Identifier":[{"@attributes":{"Source":"ORCID"},"@text":"0000-0001-8369-8800"}],"AffiliationInfo":[{"Affiliation":"Department of Pathology and Applied Neurobiology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, 465 Kajii-cho, Kawaramachi Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan. kxi14@koto.kpu-m.ac.jp."}]}]},"Language":["eng"],"GrantList":{"@attributes":{"CompleteYN":"Y"},"Grant":[{"GrantID":"18K07883","Agency":"Japan Society for the Promotion of Science","Country":""},{"GrantID":"21K07279","Agency":"Japan Society for the Promotion of Science","Country":""},{"GrantID":"20A1019","Agency":"Grants-in-Aid for Research from the National Center for Global Health and Medicine","Country":""},{"GrantID":"21A1018","Agency":"Grants-in-Aid for Research from the National Center for Global Health and Medicine","Country":""}]},"PublicationTypeList":{"PublicationType":[{"@attributes":{"UI":"D016428"},"@text":"Journal Article"}]},"ArticleDate":[{"@attributes":{"DateType":"Electronic"},"Year":"2022","Month":"01","Day":"30"}]},"MedlineJournalInfo":{"Country":"Switzerland","MedlineTA":"Cell Mol Life Sci","NlmUniqueID":"9705402","ISSNLinking":"1420-682X"},"ChemicalList":{"Chemical":[{"RegistryNumber":"0","NameOfSubstance":{"@attributes":{"UI":"C098884"},"@text":"Dtna protein, mouse"}},{"RegistryNumber":"0","NameOfSubstance":{"@attributes":{"UI":"D016189"},"@text":"Dystrophin"}},{"RegistryNumber":"0","NameOfSubstance":{"@attributes":{"UI":"D049029"},"@text":"Dystrophin-Associated Proteins"}},{"RegistryNumber":"0","NameOfSubstance":{"@attributes":{"UI":"D009479"},"@text":"Neuropeptides"}},{"RegistryNumber":"0","NameOfSubstance":{"@attributes":{"UI":"C085339"},"@text":"apo-dystrophin 1"}},{"RegistryNumber":"146888-27-9","NameOfSubstance":{"@attributes":{"UI":"D049030"},"@text":"Dystroglycans"}}]},"CitationSubset":["IM"],"MeshHeadingList":{"MeshHeading":[{"DescriptorName":{"@attributes":{"UI":"D000818","MajorTopicYN":"N"},"@text":"Animals"}},{"DescriptorName":{"@attributes":{"UI":"D001812","MajorTopicYN":"N"},"@text":"Blood-Brain Barrier"},"QualifierName":[{"@attributes":{"UI":"Q000378","MajorTopicYN":"N"},"@text":"metabolism"}]},{"DescriptorName":{"@attributes":{"UI":"D002478","MajorTopicYN":"N"},"@text":"Cells, Cultured"}},{"DescriptorName":{"@attributes":{"UI":"D049030","MajorTopicYN":"N"},"@text":"Dystroglycans"},"QualifierName":[{"@attributes":{"UI":"Q000235","MajorTopicYN":"N"},"@text":"genetics"},{"@attributes":{"UI":"Q000378","MajorTopicYN":"Y"},"@text":"metabolism"}]},{"DescriptorName":{"@attributes":{"UI":"D016189","MajorTopicYN":"N"},"@text":"Dystrophin"},"QualifierName":[{"@attributes":{"UI":"Q000235","MajorTopicYN":"N"},"@text":"genetics"},{"@attributes":{"UI":"Q000378","MajorTopicYN":"Y"},"@text":"metabolism"}]},{"DescriptorName":{"@attributes":{"UI":"D049029","MajorTopicYN":"N"},"@text":"Dystrophin-Associated Proteins"},"QualifierName":[{"@attributes":{"UI":"Q000235","MajorTopicYN":"N"},"@text":"genetics"},{"@attributes":{"UI":"Q000378","MajorTopicYN":"Y"},"@text":"metabolism"}]},{"DescriptorName":{"@attributes":{"UI":"D057809","MajorTopicYN":"N"},"@text":"HEK293 Cells"}},{"DescriptorName":{"@attributes":{"UI":"D006624","MajorTopicYN":"N"},"@text":"Hippocampus"},"QualifierName":[{"@attributes":{"UI":"Q000166","MajorTopicYN":"N"},"@text":"cytology"},{"@attributes":{"UI":"Q000378","MajorTopicYN":"N"},"@text":"metabolism"}]},{"DescriptorName":{"@attributes":{"UI":"D006801","MajorTopicYN":"N"},"@text":"Humans"}},{"DescriptorName":{"@attributes":{"UI":"D008822","MajorTopicYN":"N"},"@text":"Mice, Transgenic"}},{"DescriptorName":{"@attributes":{"UI":"D018613","MajorTopicYN":"N"},"@text":"Microscopy, Confocal"}},{"DescriptorName":{"@attributes":{"UI":"D009457","MajorTopicYN":"N"},"@text":"Neuroglia"},"QualifierName":[{"@attributes":{"UI":"Q000378","MajorTopicYN":"Y"},"@text":"metabolism"}]},{"DescriptorName":{"@attributes":{"UI":"D009474","MajorTopicYN":"N"},"@text":"Neurons"},"QualifierName":[{"@attributes":{"UI":"Q000378","MajorTopicYN":"N"},"@text":"metabolism"}]},{"DescriptorName":{"@attributes":{"UI":"D009479","MajorTopicYN":"N"},"@text":"Neuropeptides"},"QualifierName":[{"@attributes":{"UI":"Q000235","MajorTopicYN":"N"},"@text":"genetics"},{"@attributes":{"UI":"Q000378","MajorTopicYN":"Y"},"@text":"metabolism"}]},{"DescriptorName":{"@attributes":{"UI":"D011485","MajorTopicYN":"N"},"@text":"Protein Binding"}},{"DescriptorName":{"@attributes":{"UI":"D013569","MajorTopicYN":"N"},"@text":"Synapses"},"QualifierName":[{"@attributes":{"UI":"Q000378","MajorTopicYN":"Y"},"@text":"metabolism"}]}]},"KeywordList":[{"@attributes":{"Owner":"NOTNLM"},"Keyword":[{"@attributes":{"MajorTopicYN":"N"},"@text":"DMD"},{"@attributes":{"MajorTopicYN":"N"},"@text":"Dp71"},{"@attributes":{"MajorTopicYN":"N"},"@text":"Dystrophin"},{"@attributes":{"MajorTopicYN":"N"},"@text":"Glia limitans"},{"@attributes":{"MajorTopicYN":"N"},"@text":"Inhibitory postsynapse"},{"@attributes":{"MajorTopicYN":"N"},"@text":"Transgenic mouse"}]}],"CoiStatement":"The authors state no conflicts of interest."},"PubmedData":{"History":{"PubMedPubDate":[{"@attributes":{"PubStatus":"received"},"Year":"2021","Month":"11","Day":"17"},{"@attributes":{"PubStatus":"accepted"},"Year":"2022","Month":"1","Day":"14"},{"@attributes":{"PubStatus":"revised"},"Year":"2021","Month":"12","Day":"24"},{"@attributes":{"PubStatus":"entrez"},"Year":"2022","Month":"1","Day":"31","Hour":"6","Minute":"5"},{"@attributes":{"PubStatus":"pubmed"},"Year":"2022","Month":"2","Day":"1","Hour":"6","Minute":"0"},{"@attributes":{"PubStatus":"medline"},"Year":"2022","Month":"2","Day":"8","Hour":"6","Minute":"0"},{"@attributes":{"PubStatus":"pmc-release"},"Year":"2022","Month":"1","Day":"30"}]},"PublicationStatus":"epublish","ArticleIdList":{"ArticleId":[{"@attributes":{"IdType":"pubmed"},"@text":"35098363"},{"@attributes":{"IdType":"pmc"},"@text":"PMC11071725"},{"@attributes":{"IdType":"doi"},"@text":"10.1007\/s00018-022-04151-2"},{"@attributes":{"IdType":"pii"},"@text":"10.1007\/s00018-022-04151-2"}]},"ReferenceList":[{"Reference":[{"Citation":"Deburgrave 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