Mavacamten rescues increased myofilament calcium sensitivity and dysregulation of Ca2+ flux caused by thin filament hypertrophic cardiomyopathy mutations

Alexer J. Sparrow; Hugh Watkins; Matthew J. Daniels; Charles Redwood; Paul Robinson

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Mavacamten rescues increased myofilament calcium sensitivity and dysregulation of Ca2+ flux caused by thin filament hypertrophic cardiomyopathy mutations

机译：Mavacamten拯救增加丝丝钙敏感性和Ca2 +通量的缺陷引起的薄纱肥厚性心肌病变突变

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Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease, globally affecting 1 in 500 people (10). It is predominantly caused by mutations in sarcomeric proteins (20, 21), which alter Ca2+ cycling, contractility (8, 13), and myocardial energetics (21). To date there is no widely available effective treatment that acts on the primary cause of the disease, the sarcomere. Current treatments include (3-block-ers that prevent arrhythmias (18) or Ca2+ channel blockers (2) such as verapamil or diltiazem, which act to prevent diastolic dysfunction by prolonging left ventricular filling time (18); however, they do not affect the underlying altered myofilament function (5).

机译：肥厚性心肌病（HCM）是最常见的遗传性心脏病，全球影响500人（10）。它主要由糖蛋白蛋白（20,21）中的突变引起的，其改变Ca2 +循环，收缩性（8,13）和心肌能量（21）。迄今为止，没有广泛可用的有效治疗方法，可行于疾病的主要原因，Sarcomere。目前的治疗包括（3个嵌段 - 预防心律失常（18）或CA2 +通道阻滞剂（2），如维拉帕米尔或Diltiazem，其采用延长左心室填充时间（18）来防止舒张功能障碍;但是，它们不会影响底层改变了近透功能（5）。

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《American Journal of Physiology》 |2020年第2期|共8页
作者
Alexer J. Sparrow; Hugh Watkins; Matthew J. Daniels; Charles Redwood; Paul Robinson;
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Division of Cardiovascular Medicine Radcliffe Department of Medicine University of Oxford Oxford;

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正文语种 eng
中图分类人体生理学;
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1. Mavacamten rescues increased myofilament calcium sensitivity and dysregulation of Ca2+ flux caused by thin filament hypertrophic cardiomyopathy mutations [J] . Alexer J. Sparrow, Hugh Watkins, Matthew J. Daniels, American Journal of Physiology . 2020,第3Pta2期

机译：Mavacamten拯救增加丝丝钙敏感性和Ca2 +通量的缺陷引起的薄纱肥厚性心肌病变突变
2. Computational simulation of hypertrophic cardiomyopathy mutations in Troponin I: Influence of increased myofilament calcium sensitivity on isometric force, ATPase and (Ca2+)i. [J] . Kataoka A, Hemmer C, Chase PB Journal of Biomechanics . 2007,第9期

机译：肌钙蛋白I中肥厚型心肌病突变的计算模拟：肌丝钙敏感性增加对等轴测力，ATPase和（Ca2 +）i的影响。
3. Long-term rescue of a familial hypertrophic cardiomyopathy caused by a mutation in the thin filament protein, tropomyosin, via modulation of a calcium cycling protein. [J] . Gaffin RD, Pena JR, Alves MS, Journal of Molecular and Cellular Cardiology . 2011,第5期

机译：通过调节钙循环蛋白，长期拯救由细丝蛋白原肌球蛋白突变引起的家族性肥厚型心肌病。
4. In vivo loss of estrogen increases Ca2+ influx via voltage-gated calcium channels in mesenteric arterial smooth muscle cells. [D] . Fernando, Charmain Angela. 2013

机译：体内雌激素的损失通过电压门控的钙离子通道增加肠系膜动脉平滑肌细胞中的Ca2 +流入。
5. Desensitization of Myofilaments to Ca2+ as a Therapeutic Target for Hypertrophic Cardiomyopathy with Mutations in Thin Filament Proteins [O] . Marco L. Alves, Fernando A.L. Dias, Robert D. Gaffin, -1

机译：肌丝对Ca2 +的脱敏作为肥厚型心肌病的治疗靶点其细丝蛋白发生突变。
6. Long-term rescue of a familial hypertrophic cardiomyopathy caused by a mutation in the thin filament protein, tropomyosin, via modulation of a calcium cycling protein [O] . Robert D. Gaffin, James R. Peña, Marco S.L. Alves, 2011

机译：通过调节钙循环蛋白，通过调节薄纱蛋白突变引起的家族肥厚性心肌病的长期拯救

Mavacamten rescues increased myofilament calcium sensitivity and dysregulation of Ca2+ flux caused by thin filament hypertrophic cardiomyopathy mutations

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