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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
Original Article |
Different Dystrophin-like Complexes Are Expressed in Neurons and Glia
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
1.used in this paper: DMD, Duchenne muscular dystrophy; DPC, dystrophin-associated protein complex; LM, light membrane; NMDA, N-methyl d-aspartate; nNOS, neuronal nitric oxide synthase; PSD, postsynaptic density; SM, synaptic membrane
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