@article{6d83d9fc2a744fb6ad9f1fd2f73aec37,
title = "Erythrocyte spectrin peak II phosphorylation in Duchenne muscular dystrophy",
abstract = "Duchenne muscular dystrophy (DMD) is a rapidly progressive crippling disease of young boys that is inherited as an X-linked recessive trait. Previous studies have demonstrated the usefulness of erythrocyte studies in exploring membrane abnormalities in inherited muscular dystrophy. Erythrocyte spectrin peak II protein (m.w. {reversed tilde equals} 220,000) was more highly phosphorylated under initial rate conditions in DMD than in controls. The extent of peak II phosphorylation was greater in DMD erythrocytes and a Na+ stimulated peak II phosphorylation effect (Avruch and Fairbanks 1974) was not found to account for the differences between DMD and controls. The phosphorylated state of spectrin proteins in the membrane was evaluated and no differences in DMD could be measured. The maximal transfer of phosphate from [γ-32P]ATP to spectrin peak II accounts for approximately 5-10% of the total phosphate content of spectrin.",
author = "Roses, {Allen D.} and Appel, {Stanley H.}",
note = "Funding Information: Duchenne muscular dystrophy (DMD) is a rapidly progressive, crippling disease of young boys that is inherited as an X-linked recessive trait (Walton 1974). The direct analysis of muscle constituents has failed to yield significant information about the primary biochemical defect because of the presence of an extensive range of secondary effects such as increased connective tissue, fatty infiltration, and variable changes of degeneration and regeneration that are the responses of muscle to injuries This work was supported by RR-30 from the General Clinical Research Centers Program of the Division of Research Resources, National Institute of Health; 1 PO1 NS12213-01 NSPB from the National Institute of Neurological and Communicative Disorders and Stoke, National Institute of Health; and a Basil O'Connor Starter Research Grant No. 5-36 from the National Foundation March of Dimes to A.D.R. Copyright: Copyright 2014 Elsevier B.V., All rights reserved.",
year = "1976",
month = oct,
doi = "10.1016/0022-510X(76)90170-2",
language = "English (US)",
volume = "29",
pages = "185--193",
journal = "Journal of the Neurological Sciences",
issn = "0022-510X",
publisher = "Elsevier",
number = "2-4",
}