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A genetic model for the study of abnormal nerve-muscle interactions at the level of excitation-contraction coupling: the mutation muscular dysgenesis.

Authors
  • Pinçon-Raymond, M
  • García, L
  • Romey, G
  • Houenou, L
  • Lazdunski, M
  • Rieger, F
Type
Published Article
Journal
Journal de physiologie
Publication Date
Jan 01, 1990
Volume
84
Issue
1
Pages
82–87
Identifiers
PMID: 2193149
Source
Medline
License
Unknown

Abstract

Excitation-contraction in muscle fibers are coupled through a complex mechanism involving multiproteic components located at a specialized cellular site, the triadic junction. Triads in normal muscle fiber result from the apposition of sarcoplasmic reticulum citernae and T-tubule and possess strikingly organized ultrastructural elements, bridging both types of membranes, the "junctional feet". Muscular dysgenesis in the mouse is characterized by total muscle inactivity in the developing skeletal muscles due to excitation-contraction uncoupling. Triads have been found to be disorganized with no "junctional feet" and dihydropyridine (DHP) binding sites are decreased with no slow Ca2+ currents, suggesting a basic defect in the excitation-contraction coupling machinery itself. We may hypothesize that muscular dysgenesis results in a marked defect in a functional protein involved in the morphogenesis of the triad and/or directly involved in Ca2+ release for contraction.

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