基因名称(Gene Name)
Actin, Muscle Specific
蛋白名称
Actin, Muscle Specific
免疫原
Synthesized peptide derived from human Actin, Muscle Specific
特异性
This antibody detects endogenous levels of human Actin, Muscle Specific. Heat-induced epitope retrieval (HIER) Citrate buffer of pH6.0 was highly recommended as antigen repair method in paraffin secti
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
来源
Mouse, Monoclonal/IgG1, Kappa
稀释比例
IHC-p 1:100-500, WB 1:200-1000. IF 1:50-200
纯化工艺
The antibody was affinity-purified from mouse ascites by affinity-chromatography using specific immunogen.
背景
Actins are highly conserved proteins that are involved in various types of cell motility. Polymerization of globular actin (G-actin) leads to a structural filament (F-actin) in the form of a two-stranded helix. Each actin can bind to four others. The protein encoded by this gene belongs to the actin family which is comprised of three main groups of actin isoforms, alpha, beta, and gamma. The alpha actins are found in muscle tissues and are a major constituent of the contractile apparatus. Defects in this gene have been associated with idiopathic dilated cardiomyopathy (IDC) and familial hypertrophic cardiomyopathy (FHC). [provided by RefSeq, Jul 2008],
功能
disease:Defects in ACTC1 are the cause of cardiomyopathy dilated type 1R (CMD1R) [MIM:102540]. 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.,disease:Defects in ACTC1 are the cause of cardiomyopathy familial hypertrophic type 11 (CMH11) [MIM:612098]. 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.,function:Actins are highly conserv
