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The Journal of Cell Biology, Vol 108, 503-510, Copyright © 1989 by The Rockefeller University Press
ARTICLES |
EP Hoffman, SC Watkins, HS Slayter and LM Kunkel
Division of Genetics, Children's Hospital, Boston, Massachusetts 02115.
We have characterized a protein immunologically related to dystrophin, the protein product of the Duchenne muscular dystrophy gene. We identify this related protein as a fast-twitch glycolytic isoform (mouse extensor digitorum longus-specific) of myofibrillar alpha- actinin. This specific isoform of alpha-actinin exhibits a more restricted pattern of expression in skeletal muscle than fast-twitch- specific isoforms of both myosin and Ca2+-ATPase. Our results provide evidence that dystrophin and myofibrillar alpha-actinin are related proteins, reinforcing the previous data concerning the sequence homologies noted between nonmuscle cytoskeletal alpha-actinin and dystrophin. In addition, we describe the first antisera directed against a specific myofibrillar skeletal muscle isoform of alpha- actinin.
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