LQT4 gene: the "missing" ankyrin.
Mutations in ion channels have been implicated in the formation of long QT syndrome (LQTS). However, Mohler et al. have recently uncovered a role for ankyrin-B, a non-ion channel protein, in type IV LQTS. Calcium signalling is altered, and the functions of several channels and pumps that normally interact with wild-type ankyrin-B are impaired in the presence of mutant ankyrin-B. The authors suggest that by disrupting the functions of these channels, a new mechanism has been uncovered that can lead to cardiac myopathy.
Center for Molecular Genetics, Department of Molecular Cardiology, Lerner Research Institute, The Cleveland Clinic Foundation, Cleveland, OH, 44195, USA.
Sandro
S
Yong
Xiaoli
X
Tian
Qing
Q
Wang
D000200:Action Potentials; D000818:Animals; D017487:Ankyrins / Q000235:genetics*; D004562:Electrocardiography; D006321:Heart / Q000502:physiology; D006801:Humans; D008133:Long QT Syndrome / Q000235:genetics*; D008954:Models, Biological
D017487:Ankyrins