Human Molecular Genetics, Vol 5, 1299-1303, Copyright © 1996 by Oxford University Press
DS Greenberg, Y Schatz, Z Levy, P Pizzo, D Yaffe and U Nudel
Duchenne muscular dystrophy (DMD) is a progressive degenerative lethal
muscle disease. A significant proportion of DMD affected children suffer
also from mental retardation. The rod shaped protein, dystrophin, which is
absent from or defective in the muscle of DMD patients, binds to a number
of membrane associated proteins (known collectively as dystrophin
associated proteins [DAPs]). The levels of DAPs is greatly reduced in the
muscle of DMD patients and mdx mice, which lack dystrophin. In addition to
dystrophin isoforms, the DMD gene codes also for several smaller proteins.
One of the small proteins, Dp71, is expressed in most or all non-muscle
tissues and is the major DMD gene product in the brain. The function of the
small DMD gene products is unknown. Here we show that mutant mice which do
not express the smaller non-muscle products of the DMD gene have a reduced
level of DAPs in their brain. This suggests that Dp71 is important for the
formation and/or stabilization of a DAPs complex in brain.
ARTICLES
Reduced levels of dystrophin associated proteins in the brains of mice deficient for Dp71
Department of Molecular Cell Biology, Weizmann Institute of Science, Rehovot, Israel.
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