本公司提供科研MYH7肌球蛋白重链7抗体,抗体质量可靠,订购MYH7肌球蛋白重链7抗体请联系在线客服或者销售人员。
抗体参数如下>>>>
中文名称:肌球蛋白重链7抗体
英文名称:Anti-MYH7
货号:bs-9862R
抗体来源:兔
克隆类型:多克隆
蛋白分子量:predicted molecular weight: 213kDa
纯化方法:affinity purified by Protein A
交叉反应:hu, mo, rat, chk, hrs
测试应用:ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500
(石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
产品背景介绍:Myosin heavy chains are ubiquitous Actin-based motor proteins that convert the chemical energy derived from ATP hydrolysis into the mechanical energy that drives diverse motile processes in eukaryotic cells, including cytokinesis, vesicular transport and cellular locomotion. Muscle myosin is a heterohexamer consisting of two myosin heavy chains and two associated nonidentical pairs of myosin light chains. The seven myosin heavy chain isoforms that predominate in mammalian skeletal muscles include two developmental isoforms, MHC-embryonic (MYH3) and MHC-perinatal (MYH8); three adult skeletal muscle isoforms, MHC IIa (MYH2), MHC IIb (MYH4) and MHC IIx/d (MYH1); and MHC-∫/slow (MYH7 or MHC-∫), which is also expressed in cardiac muscle. Research indicates that mutations of the MYH7 gene causes hypertrophic cardiomyopathy.Function : Muscle contraction.Subunit : Muscle myosin is a hexameric protein that consists of 2 heavy chain subunits (MHC), 2 alkali light chain subunits (MLC) and 2 regulatory light chain subunits (MLC-2).Subcellular Location : Cytoplasm, myofibril. Note=Thick filaments of the myofibrils.DISEASE : Defects in MYH7 are the cause of cardiomyopathy familial hypertrophic type 1 (CMH1) [MIM:192600]. Familial hypertrophic cardiomyopathy is a hereditary heart disorder characterized by ventricular hypertrophy, which is usually asymmetric and often involves the interventricular septum. The symptoms include dyspnea, syncope, collapse, palpitations, and chest pain. They can be readily provoked by exercise. The disorder has inter- and intrafamilial variability ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death.Defects in MYH7 are the cause of myopathy myosin storage (MYOMS) [MIM:608358]. In this disorder, muscle biopsy shows type 1 fiber predominance and increased interstitial fat and connective tissue. Inclusion bodies consisting of the beta cardiac myosin heavy chain are present in the majority of type 1 fibers, but not in type 2 fibers.Similarity : Contains 1 IQ domain.Contains 1 myosin head-like domain.Defects in MYH7 are the cause of scapuloperoneal myopathy MYH7-related (SPMM) [MIM:181430]; also known as scapuloperoneal syndrome myopathic type. SPMM is a progressive muscular atrophia beginning in the lower legs and affecting the shoulder region earlier and more severely than distal arm.Defects in MYH7 are a cause of cardiomyopathy dilated type 1S (CMD1S) [MIM:613426]. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.Defects in MYH7 are the cause of myopathy distal type 1 (MPD1) [MIM:160500]. MPD1 is a muscular disorder characterized by early-onset selective weakness of the great toe and ankle dorsiflexors, followed by weakness of the finger extensors. Mild proximal weakness occasionally develops years later after the onset of the disease.