Leaky ryanodine receptors contribute to diaphragmatic weakness during mechanical ventilation.
Matecki, Stefan; Dridi, Haikel; Jung, Boris; Saint, Nathalie; Reiken, Steven R; Scheuermann, Valérie; Mrozek, Ségolène; Santulli, Gaetano; Umanskaya, Alisa; Petrof, Basil J; Jaber, Samir; Marks, Andrew R; Lacampagne, Alain.
Proc Natl Acad Sci U S A
; 113(32): 9069-74, 2016 08 09.
Artigo em Inglês | MEDLINE | ID: mdl-27457930
A novel ryanodine receptor mutation linked to sudden death increases sensitivity to cytosolic calcium.
Insights into the gating mechanism of the ryanodine-modified human cardiac Ca2+-release channel (ryanodine receptor 2).
Leaky RyR2 channels unleash a brainstem spreading depolarization mechanism of sudden cardiac death.
Distinctive malfunctions of calmodulin mutations associated with heart RyR2-mediated arrhythmic disease.
Receptor de rianodina, fuga de calcio y arritmias.
[FK506 binding protein (FKBP12/12.6)].
Enhanced store overload-induced Ca2+ release and channel sensitivity to luminal Ca2+ activation are common defects of RyR2 mutations linked to ventricular tachycardia and sudden death.
Characterization and temporal development of cores in a mouse model of malignant hyperthermia.
Techniques and methodologies to study the ryanodine receptor at the molecular, subcellular and cellular level.