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Alpha-dystrobrevin is a dystrophin-related and -associated protein that is involved in synapse maturation and is required for normal muscle function. There are three protein isoforms in skeletal muscle, alpha-dystrobrevin-1, -2, and -3 that are encoded by the single alpha-dystrobrevin gene. To understand the role of these proteins in muscle we have investigated the localisation and transcript distribution of the different alpha-dystrobrevin isoforms. Alpha-dystrobrevin-1 and -2 are concentrated at the neuromuscular junction and are both recruited into agrin-induced acetylcholine receptor clusters in cultured myotubes. We also demonstrate that all alpha-dystrobrevin mRNAs are transcribed from a single promoter in skeletal muscle. However, only transcripts encoding alpha-dystrobrevin-1 are preferentially accumulated at postsynaptic sites. These data suggest that the synaptic accumulation of alpha-dystrobrevin-1 mRNA occurs posttranscriptionally, identifying a novel mechanism for synaptic gene expression. Taken together, these results indicate that different isoforms possess distinct roles in synapse formation and possibly in the pathogenesis of muscular dystrophy.

Original publication




Journal article


Mol Cell Neurosci

Publication Date





127 - 140


Agrin, Animals, Cell Line, Cytoskeletal Proteins, Dystrophin-Associated Proteins, Gene Expression Regulation, Membrane Proteins, Mice, Mice, Inbred C57BL, Mice, Inbred mdx, Muscle, Skeletal, Promoter Regions, Genetic, Protein Isoforms, RNA, Messenger, Receptor Aggregation, Receptors, Cholinergic, Synapses