Specific absence of the α1 subunit of the dihydropyridine receptor in mice with muscular dysgenesis

C. M. Knudson, N. Chaudhari, A. H. Sharp, J. A. Powell, K. G. Beam, K. P. Campbell

Research output: Contribution to journalArticle

100 Scopus citations

Abstract

Muscular dysgenesis is a lethal mutation in mice that results in a complete absence of skeletal muscle contraction due to the failure of depolarization of the transverse tubular membrane to trigger calcium release from the sarcoplasmic reticulum. In order to determine whether the defect in muscular dysgenesis leads to a specific loss of one of the components of excitation-contraction coupling or to a generalized loss of all components of excitation-contraction coupling, we have analyzed skeletal muscle from control and dysgenic mice for the sarcoplasmic reticulum and transverse tubular proteins which are believed to function in excitation-contraction coupling. We report that the proteins involved in sarcoplasmic reticulum calcium transport, storage, and release ((Ca2+ + Mg2+)-ATPase, calsequestrin, and calcium release channel) are present in dysgenic muscle. Also present in dysgenic muscle is the 175/150-kDa glycoprotein subunit (α2) of the dihydropyridine receptor. However, the 170-kDa dihydropyridine binding subunit (α1) of the dihydropyridine receptor is absent in dysgenic muscle. These results suggest that the specific absence of the α1 subunit of the dihydropyridine receptor is responsible for the defects in muscular dysgenesis and that the α1 subunit of the dihydropyridine receptor is essential for excitation-contraction coupling in skeletal muscle.

Original languageEnglish (US)
Pages (from-to)1345-1348
Number of pages4
JournalJournal of Biological Chemistry
Volume264
Issue number3
StatePublished - Jan 1 1989
Externally publishedYes

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Cell Biology

Fingerprint Dive into the research topics of 'Specific absence of the α<sub>1</sub> subunit of the dihydropyridine receptor in mice with muscular dysgenesis'. Together they form a unique fingerprint.

  • Cite this

    Knudson, C. M., Chaudhari, N., Sharp, A. H., Powell, J. A., Beam, K. G., & Campbell, K. P. (1989). Specific absence of the α1 subunit of the dihydropyridine receptor in mice with muscular dysgenesis. Journal of Biological Chemistry, 264(3), 1345-1348.