Article
Drastic Ca2+ sensitization of myofilament associated with a small structural change in troponin I in inherited restrictive cardiomyopathy.
Biochemical and biophysical research communications - 23 Dec 2005
Yumoto Fumiaki, Lu Qun-Wei, Morimoto Sachio, Tanaka Hiroyuki, Kono Naoko, Nagata Koji, Ojima Takao, Takahashi-Yanaga Fumi, Miwa Yoshikazu, Sasaguri Toshiyuki, Nishita Kiyoyoshi, Tanokura Masaru, Ohtsuki Iwao
Abstract excerpt
Six missense mutations in human cardiac troponin I (cTnI) were recently found to cause restrictive cardiomyopathy (RCM). We have bacterially expressed and purified these human cTnI mutants and examined their functional and structural consequences. Inserting the human cTnI into skinned cardiac muscle fibers showed that these mutations had much greater Ca2+-sensitizing effects on force generation than the cTnI...
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