نتایج جستجو برای: calcium channelopathy

تعداد نتایج: 167790  

2009
Sang-Chan Lee Hyang-Sook Kim Yeong-Eun Park Young-Chul Choi Kyu-Hyun Park Dae-Seong Kim

BACKGROUND AND PURPOSE Mutations of the skeletal muscle sodium channel gene SCN4A, which is located on chromosome 17q23-25, are associated with various neuromuscular disorders that are labeled collectively as skeletal muscle sodium channelopathy. These disorders include hyperkalemic periodic paralysis (HYPP), hypokalemic periodic paralysis, paramyotonia congenita (PMC), potassium-aggravated myo...

Journal: :The Journal of clinical investigation 2015
David S Park Marina Cerrone Gregory Morley Carolina Vasquez Steven Fowler Nian Liu Scott A Bernstein Fang-Yu Liu Jie Zhang Christopher S Rogers Silvia G Priori Larry A Chinitz Glenn I Fishman

SCN5A encodes the α subunit of the major cardiac sodium channel Na(V)1.5. Mutations in SCN5A are associated with conduction disease and ventricular fibrillation (VF); however, the mechanisms that link loss of sodium channel function to arrhythmic instability remain unresolved. Here, we generated a large-animal model of a human cardiac sodium channelopathy in pigs, which have cardiac structure a...

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