نتایج جستجو برای: calcium channelopathy

تعداد نتایج: 167790  

Journal: :Investigative ophthalmology & visual science 2006
Katharina A Wycisk Birgit Budde Silke Feil Sergej Skosyrski Francesca Buzzi John Neidhardt Esther Glaus Peter Nürnberg Klaus Ruether Wolfgang Berger

PURPOSE In a spontaneous mutant substrain of C57BL/10 mice, severely affected retinal ribbon-type synapses have been described. The retinopathy was accompanied by a substantial loss in the activities of the second-order neurons. Rod photoreceptor responses were maintained with reduced amplitude, whereas cone activities were absent. This study was conducted to identify the genetic defect underly...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2002
Hui Sun Takashi Tsunenari King-Wai Yau Jeremy Nathans

Vitelliform macular dystrophy (VMD/Best disease; MIM*153700) is an early-onset autosomal dominant disorder in which accumulation of lipofuscin-like material within and beneath the retinal pigment epithelium is associated with a progressive loss of central vision. Bestrophin, the protein product of the VMD gene, has four predicted transmembrane domains. There are multiple bestrophin homologues i...

2017
Xinlin Zhang Jun Xie Suhui Zhu Yuhan Chen Lian Wang Biao Xu

Hypertrophic cardiomyopathy (HCM) is a highly heterogeneous disease displaying considerable interfamilial and intrafamilial phenotypic variation, including disease severity, age of onset, and disease progression. This poorly understood variance raises the possibility of genetic modifier effects, particularly in MYBPC3-associated HCM.In a large consanguineous Chinese HCM family, we identified 8 ...

2011
Ronald J. Kanter Ryan Pfeiffer Dan Hu Héctor Barajas-Martinez Michael P. Carboni

Background—Brugada syndrome is a potentially serious channelopathy that usually presents in adulthood and has only rarely been described in infancy. In the absence of metabolic or structural cardiac disease, rapid ventricular tachycardia ( 200 bpm) and primary cardiac conduction disease are uncommon in infancy. We hypothesized that infants having rapid ventricular tachycardia and conduction abn...

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