Essential role of TRPV2 ion channel in the sensitivity of dystrophic muscle to eccentric contractions.
Duchenne myopathy is a lethal disease due to the absence of dystrophin, a cytoskeletal protein. Muscles from dystrophin-deficient mice (mdx) typically present an exaggerated susceptibility to eccentric work characterized by an important force drop and an increased membrane permeability consecutive to repeated lengthening contractions. The present study shows that mdx muscles are largely protected from eccentric work-induced damage by overexpressing a dominant negative mutant of TRPV2 ion channel. This observation points out the role of TRPV2 channel in the physiopathology of Duchenne muscular dystrophy.
Laboratory of Cell Physiology, Institute of Neurosciences, Université catholique de Louvain, 55/40 av Hippocrate, 1200 Brussels, Belgium.
Nadège
N
Zanou
Yuko
Y
Iwata
Olivier
O
Schakman
Jean
J
Lebacq
Shigeo
S
Wakabayashi
Philippe
P
Gailly
D000818:Animals; D002121:Calcium Channel Blockers / Q000494:pharmacology; D015220:Calcium Channels / Q000235:genetics / Q000502:physiology*; D002463:Cell Membrane Permeability / Q000187:drug effects; D005260:Female; D007093:Imidazoles / Q000494:pharmacology; D066298:In Vitro Techniques; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D008822:Mice, Transgenic; D009119:Muscle Contraction / Q000187:drug effects / Q000502:physiology*; D018482:Muscle, Skeletal / Q000503:physiopathology; D009137:Muscular Dystrophy, Animal / Q000503:physiopathology*; D020388:Muscular Dystrophy, Duchenne / Q000503:physiopathology*; D012508:Sarcolemma / Q000502:physiology; D050916:TRPV Cation Channels / Q000235:genetics / Q000502:physiology*
D002121:Calcium Channel Blockers; D015220:Calcium Channels; D007093:Imidazoles; D050916:TRPV Cation Channels; C485717:Trpv2 protein, mouse; C063159:1-(2-(3-(4-methoxyphenyl)propoxy)-4-methoxyphenylethyl)-1H-imidazole