产品名称
MYBPC3 Rabbit Polyclonal Antibody
别名
Myosin-binding protein C, cardiac-type (Cardiac MyBP-C) (C-protein, cardiac muscle isoform)
蛋白名称
Myosin-binding protein C, cardiac-type (Cardiac MyBP-C) (C-protein, cardiac muscle isoform)
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=4607
Human Swissprot No.
Q14896
Human Swissprot Link
http://www.uniprot.org/uniprotkb/Q14896/entry
Mouse Swissprot No.
O70468
Mouse Swissprot Link
http://www.uniprot.org/uniprot/O70468
免疫原
Synthesized peptide derived from human MYBPC3 Polyclonal
特异性
This antibody detects endogenous levels of MYBPC3.
稀释度
WB 1:500-2000;IHC-p 1:50-300
宿主
Polyclonal, Rabbit,IgG
背景介绍
MYBPC3 encodes the cardiac isoform of myosin-binding protein C. Myosin-binding protein C is a myosin-associated protein found in the cross-bridge-bearing zone (C region) of A bands in striated muscle. MYBPC3, the cardiac isoform, is expressed exclussively in heart muscle. Regulatory phosphorylation of the cardiac isoform in vivo by cAMP-dependent protein kinase (PKA) upon adrenergic stimulation may be linked to modulation of cardiac contraction. Mutations in MYBPC3 are one cause of familial hypertrophic cardiomyopathy. [provided by RefSeq, Jul 2008],
组织表达
Donated clones,Heart,Pooled,
细胞定位
cytosol,striated muscle myosin thick filament,C zone,sarcomere,A band,
信号通路
Hypertrophic cardiomyopathy (HCM);Dilated cardiomyopathy;
功能
disease:Defects in MYBPC3 are the cause of cardiomyopathy familial hypertrophic type 4 (CMH4) [MIM:115197]. 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:Thick filament-associated protein located in the crossbridge region of vertebrate striated muscle a bands. In vitro it binds MHC, F-actin and native thin filaments, and modifies the activity of actin-activated myosin ATPase. It may modulate muscle contraction or may play a more structural role.,PTM:Substrate for phosphorylation by PKA and PKC. Reversible phosphorylation appears to modulate contraction.,similarity:Belongs to the immunoglobulin superfamily. MyBP family.,similarity:Contains 3 fibronectin type-III domains.,similarity:Contains 7 Ig-like C2-type (immunoglobulin-like) domains.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.