Exogenous Dp71 restores the levels of dystrophin associated proteins but does not alleviate muscle damage in mdx mice.
Dp71 is a non-muscle product of the Duchenne muscular dystrophy gene. It consists of the cysteine-rich and C-terminal domains of dystrophin. We have generated transgenic mdx mice which do not have dystrophin but express Dp71 in their muscle. In these mice, Dp71 was localized to the plasma membrane and restored normal levels of dystrophin associated proteins (DAPs), indicating that Dp71 is capable of interacting with the DAPs in a similar manner to dystrophin. However, the presence of Dp71 and DAPs in the muscle fibres of mdx mice was not sufficient to alleviate symptoms of muscle degeneration.
Greenberg
D S
DS
Department of Cell Biology, Weizmann Institute of Science, Rehovot, Israel.
Sunada
Y
Y
Campbell
K P
KP
Yaffe
D
D
Nudel
U
U
D000818:Animals; D004195:Disease Models, Animal; D016189:Dystrophin / Q000031:analogs & derivatives* / Q000378:metabolism* / Q000494:pharmacology; D005260:Female; D008297:Male; D051379:Mice; D008822:Mice, Transgenic; D009124:Muscle Proteins / Q000378:metabolism*; D018482:Muscle, Skeletal / Q000473:pathology; D009136:Muscular Dystrophies / Q000235:genetics / Q000378:metabolism* / Q000473:pathology
D016189:Dystrophin; D009124:Muscle Proteins; C085339:apo-dystrophin 1