Acetylcholine receptor ε-subunit deletion causes muscle weakness and atrophy in juvenile and adult mice

V. Witzemann, H. Schwarz, M. Koenen, C. Berberich, A. Villarroel, A. Wernig, H. R. Brenner, B. Sakmann

Producción científica: Contribución a una revistaArtículorevisión exhaustiva

93 Citas (Scopus)

Resumen

In mammalian muscle a postnatal switch in functional properties of neuromuscular transmission occurs when miniature end plate currents become shorter and the conductance and Ca2+ permeability of end plate channels increases. These changes are due to replacement during early neonatal development of the γ-subunit of the fetal acetylcholine receptor (AChR) by the ε-subunit. The long-term functional consequences of this switch for neuromuscular transmission and motor behavior of the animal remained elusive. We report that deletion of the ε-subunit gene caused in homozygous mutant mice the persistence of γ-subunit gene expression in juvenile and adult animals. Neuromuscular transmission in these animals is based on fetal type AChRs present in the end plate at reduced density. Impaired neuromuscular transmission, progressive muscle weakness, and atrophy caused premature death 2 to 3 months after birth. The results demonstrate that postnatal incorporation into the end plate of ε-subunit containing AChRs is essential for normal development of skeletal muscle.

Idioma originalEnglish
Páginas (desde-hasta)13286-13291
Número de páginas6
PublicaciónProceedings of the National Academy of Sciences of the United States of America
Volumen93
N.º23
DOI
EstadoPublished - nov. 12 1996
Publicado de forma externa

ASJC Scopus Subject Areas

  • General

Huella

Profundice en los temas de investigación de 'Acetylcholine receptor ε-subunit deletion causes muscle weakness and atrophy in juvenile and adult mice'. En conjunto forman una huella única.

Citar esto