Erythrocyte spectrin peak II phosphorylation in Duchenne muscular dystrophy.

Published

Journal Article

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 inheritied muscular dystrophy. Erythrocyte spectrin peak II protein (m.w. equivalent to 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 differences in DMD could be measured. The maximal transfer of phosphate from [gamma-32P]ATP to spectrin peak II accounts for approximately 5-10% of the total phosphate content of spectrin.

Full Text

Cited Authors

  • Roses, AD; Appel, SH

Published Date

  • October 1, 1976

Published In

Volume / Issue

  • 29 / 2-4

Start / End Page

  • 185 - 193

PubMed ID

  • 978207

Pubmed Central ID

  • 978207

Electronic International Standard Serial Number (EISSN)

  • 1878-5883

International Standard Serial Number (ISSN)

  • 0022-510X

Digital Object Identifier (DOI)

  • 10.1016/0022-510x(76)90170-2

Language

  • eng