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© The Rockefeller University Press, 0021-9525/1999//645 $5.00
The Journal of Cell Biology, Volume 147, Number 3, , 1999 645-658

Different Dystrophin-like Complexes Are Expressed in Neurons and Glia

^a Department of Human Anatomy and Genetics, University of Oxford, Oxford OX1 3QX, United Kingdom
^b Department of Cell Biology and Anatomy, Genes and Development Research Group, University of Calgary Faculty of Medicine, Alberta T2N 4N1, Canada
^c Division of Biochemistry, School of Biological Sciences, Royal Holloway and Bedford New College, University of London, Egham, Surrey TW20 0EX, United Kingdom
Department of Human Anatomy and Genetics, University of Oxford, South Parks Road, Oxford, OX1 3QX, UK.44 1865 272 18344 1865 272 183

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. β-Dystrobrevin is found in neurons and is highly enriched in postsynaptic densities (PSDs). Furthermore, β-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 β-dystrobrevin are differentially distributed in the brain where they form separate DPC-like complexes.

Key Words: dystrobrevin • dystrophin • synapse • postsynaptic density • astrocyte

© 1999 The Rockefeller University Press

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