title: Expression of alpha-dystrobrevin in blood-tissue barriers: sub-cellular localisation and molecular characterisation in normal and dystrophic mice.
abstract: The alpha- and beta-dystrobrevins (DBs) belong to a family of dystrophin-related and dystrophin-associated proteins that are members of the dystrophin-associated protein complex (DAPC). This complex provides a link between the cytoskeleton and the extracellular matrix or other cells. However, specific functions of the two dystrobrevins remain largely unknown, with alpha-DB being believed to have a role mainly in skeletal muscle. Here, we describe previously unknown expression patterns and the localisation and molecular characteristics of alpha-DB isoforms in non-muscle mouse tissues. We demonstrate a highly specific sub-cellular distribution of alpha-DB in organs forming blood-tissue barriers. We show alpha-DB expression and localisation in testicular Sertoli cells, stomach and respiratory epithelia and provide electron-microscopic evidence for its immunolocalisation in these cells and in the central nervous system. Moreover, we present the molecular characterisation of alpha-DB transcript in these tissues and provide evidence for a distinct heterogeneity of associations between alpha-DB and dystrophins and utrophin in normal and dystrophic non-muscle tissues. Together, our results indicate that alpha-DB, in addition to its role in skeletal muscle, may also be required for the proper function of specific non-muscle tissues and that disruption of DAPC might lead to tissue-blood barrier abnormalities.
authors.affiliation: Molecular Medicine, Institute of Biomedical and Biomolecular Sciences, School of Pharmacy and Biomedical Sciences, University of Portsmouth, St. Michael's Building, White Swan Road, Portsmouth, UK.
authors.forename: Chun Fu
authors.initials: CF
authors.lastname: Lien
authors.forename: Diana
authors.initials: D
authors.lastname: Hazai
authors.forename: Davy
authors.initials: D
authors.lastname: Yeung
authors.forename: Juraini
authors.initials: J
authors.lastname: Tan
authors.forename: Ernst-Martin
authors.initials: EM
authors.lastname: Füchtbauer
authors.forename: Veronika
authors.initials: V
authors.lastname: Jancsik
authors.forename: Dariusz C
authors.initials: DC
authors.lastname: Górecki
mesh_terms: D000818:Animals; D015824:Blood-Air Barrier / Q000378:metabolism / Q000648:ultrastructure; D001812:Blood-Brain Barrier / Q000378:metabolism / Q000648:ultrastructure; D001813:Blood-Retinal Barrier / Q000378:metabolism / Q000648:ultrastructure; D001814:Blood-Testis Barrier / Q000378:metabolism / Q000648:ultrastructure; D004195:Disease Models, Animal; D049029:Dystrophin-Associated Proteins / Q000235:genetics / Q000378:metabolism*; D004848:Epithelium / Q000378:metabolism* / Q000648:ultrastructure; D019084:Fluorescent Antibody Technique, Indirect; D005753:Gastric Mucosa / Q000378:metabolism* / Q000648:ultrastructure; D015870:Gene Expression; D020868:Gene Silencing; D007124:Immunoenzyme Techniques; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D018345:Mice, Knockout; D020388:Muscular Dystrophy, Duchenne / Q000378:metabolism* / Q000473:pathology; D012333:RNA, Messenger / Q000378:metabolism; D020545:Respiratory Mucosa / Q000378:metabolism / Q000648:ultrastructure; D012708:Sertoli Cells / Q000378:metabolism* / Q000648:ultrastructure
chemical_list: D049029:Dystrophin-Associated Proteins; D012333:RNA, Messenger; C098885:dystrobrevin