免疫原
Synthetic peptide from human protein at AA range: 1871-1920
特异性
The antibody detects endogenous MYH6/MYH7
来源
Polyclonal, Rabbit,IgG
组成(Formulation)
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
IHC-p 1:50-200, ELISA 1:10000-20000. IF 1:50-200
纯化工艺(Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
背景(Background)
Cardiac muscle myosin is a hexamer consisting of two heavy chain subunits, two light chain subunits, and two regulatory subunits. This gene encodes the alpha heavy chain subunit of cardiac myosin. The gene is located ~4kb downstream of the gene encoding the beta heavy chain subunit of cardiac myosin. Mutations in this gene cause familial hypertrophic cardiomyopathy and atrial septal defect 3. [provided by RefSeq, Mar 2010],
功能
disease:Defects in MYH6 are a cause of cardiomyopathy familial hypertrophic (CMH) [MIM:192600]; also designated FHC or HCM. 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.,disease:Defects in MYH6 are the cause of atrial septal defect type 3 (ASD3) [MIM:160710]. ASD3 is a congenital heart malformation characterized by incomplete closure of the wall between the atria resulting in blood flow from the left to the right atria.,domain:The rodlike tail sequence is highly repetitive, showing
基因名称(Gene Name)
MYH6/7 MYHCA/B
蛋白名称
Myosin-6 (Myosin heavy chain 6) (Myosin heavy chain, cardiac muscle alpha isoform) (MyHC-alpha) Myosin-7B (Antigen MLAA-21) (Myosin cardiac muscle beta chain) (Myosin heavy chain 7B, cardiac muscle be
Fields
>>cGMP-PKG signaling pathway;>>Cardiac muscle contraction;>>Adrenergic signaling in cardiomyocytes;>>Thyroid hormone signaling pathway;>>Hypertrophic cardiomyopathy;>>Dilated cardiomyopathy;>>Viral myocarditis
细胞定位
Cytoplasm, myofibril. Thick filaments of the myofibrils.