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Original Article |
Correspondence to: Derek J. Blake, Department of Human Anatomy and Genetics, University of Oxford, South Parks Road, Oxford, OX1 3QX, UK. Tel:44 1865 272 183 Fax:44 1865 272 183 E-mail:dblake{at}enterprise.molbiol.ox.ac.uk.
Duchenne muscular dystrophy is a fatal muscle disease that is often associated with cognitive impairment. Accordingly, dystrophin is found at the muscle sarcolemma and at postsynaptic sites in neurons. In muscle, dystrophin forms part of a membrane-spanning complex, the dystrophin-associated protein complex (DPC). Whereas the composition of the DPC in muscle is well documented, the existence of a similar complex in brain remains largely unknown. To determine the composition of DPC-like complexes in brain, we have examined the molecular associations and distribution of the dystrobrevins, a widely expressed family of dystrophin-associated proteins, some of which are components of the muscle DPC. ß-Dystrobrevin is found in neurons and is highly enriched in postsynaptic densities (PSDs). Furthermore, ß-dystrobrevin forms a specific complex with dystrophin and syntrophin. By contrast, 
-dystrobrevin-1 is found in perivascular astrocytes and Bergmann glia, and is not PSD-enriched. -Dystrobrevin-1 is associated with Dp71, utrophin, and syntrophin. In the brains of mice that lack dystrophin and Dp71, the dystrobrevin–syntrophin complexes are still formed, whereas in dystrophin-deficient muscle, the assembly of the DPC is disrupted. Thus, despite the similarity in primary sequence, - and ß-dystrobrevin are differentially distributed in the brain where they form separate DPC-like complexes.
Key Words: dystrobrevin, dystrophin, synapse, postsynaptic density, astrocyte
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