Cold-induced disruption of Na+channel slow inactivation underlies paralysis in highly thermosensitive paramyotonia
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چکیده
منابع مشابه
Slow inactivation of cardiac L-type Ca21 channel induced by cold acclimation of guinea pig
Takagi, Shuichi, Yasuki Kihara, Shigetake Sasayama, and Tamotsu Mitsuiye. Slow inactivation of cardiac L-type Ca21 channel induced by cold acclimation of guinea pig. Am. J. Physiol. 274 (Regulatory Integrative Comp. Physiol. 43): R348–R356, 1998.—Whole cell L-type Ca21 current was recorded in ventricular myocytes dissociated from guinea pigs that were bred at ambient temperatures ranging betwee...
متن کاملSlow inactivation of cardiac L-type Ca2+ channel induced by cold acclimation of guinea pig.
Whole cell L-type Ca2+ current was recorded in ventricular myocytes dissociated from guinea pigs that were bred at ambient temperatures ranging between daily averages of 4 and 29°C. The dynamic voltage range of inactivation, as measured using 400-ms conditioning pulses and a holding potential of -40 mV, extended from -50 to -20 mV in myocytes prepared in summer. In winter, the inactivation curv...
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Emotion-induced blindness refers to impaired awareness of stimuli appearing in the temporal wake of an emotionally arousing stimulus (S. B. Most, Chun, Widders, & Zald, 2005). In previous emotion-induced blindness experiments, participants withheld target responses until the end of a rapid stream of stimuli, even though each target appeared in the middle of the stream. The resulting interval be...
متن کاملAltered fast and slow inactivation of the N440K Nav1.4 mutant in a periodic paralysis syndrome.
OBJECTIVE To electrophysiologically characterize the Na(v)1.4 mutant N440K found in a Korean family with a syndrome combining symptoms of paramyotonia congenita, hyperkalemic periodic paralysis, and potassium-aggravated myotonia. METHODS We characterized transiently expressed wild-type and mutant Na(v)1.4 using whole-cell voltage-clamp analysis. RESULTS N440K produced a significant depolari...
متن کاملA double mutation in families with periodic paralysis defines new aspects of sodium channel slow inactivation.
Hyperkalemic periodic paralysis (HyperKPP) is an autosomal dominant skeletal muscle disorder caused by single mutations in the SCN4A gene, encoding the human skeletal muscle voltage-gated Na(+) channel. We have now identified one allele with two novel mutations occurring simultaneously in the SCN4A gene. These mutations are found in two distinct families that had symptoms of periodic paralysis ...
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ژورنال
عنوان ژورنال: The Journal of Physiology
سال: 2009
ISSN: 0022-3751
DOI: 10.1113/jphysiol.2008.165787