心脏肌球蛋白结合蛋白抗体
规格：1尘驳/1尘濒
英文名： MYBPC3
别名： C protein cardiac muscle isoform; cardiac muscle isoform; cardiac-type; C-protein; Cardiac MyBP C; Cardiac MyBP-C; Cardiac myosin binding protein C; MYBP C; MYBPC; MYBPC3; Myosin binding protein C car
分子量： 141kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human MYBPC3
交叉反应：Human, Mouse, Rat, Cow, Horse, Rabbit,
细胞定位：
心脏肌球蛋白结合蛋白抗体产物介绍：background: MYBPC3 encodes the cardiac isoform of the thick-filament myosin-binding protein C. It is found in the crossbridge-bearing zone (C region) of A bands in vertebrate striated muscle. Regulatory phosphorylation of MYBPC3 by cAMP-dependent protein kinase (PKA) upon adrenergic stimulation may be linked to modulation of cardiac contraction. MYBPC3 binds F-Actin, MHC and native thin filaments, and modifies the activity of Actin-activated myosin ATPase. Mutations in the MYBPC3 gene lead mainly to truncation of the protein, which results in one cause of familial hypertrophic cardiomyopathy type 4 (CMH4), a heart disorder characterized by ventricular hypertrophy, which often involves the interventricular septum and is usually asymmetric. The MYBPC3 gene maps to chromosome 11p11.2. 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. Post-translational modifications: Substrate for phosphorylation by PKA and PKC. Reversible phosphorylation appears to modulate contraction (By similarity). DISEASE: Defects in MYBPC3 are the cause of familial hypertrophic cardiomyopathy 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心脏肌球蛋白结合蛋白抗体 disorder has inter- and intrafamilial variability ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death. Similarity: Belongs to the immunoglobulin superfamily. MyBP family. Contains 3 fibronectin type-III domains. Contains 7 Ig-like C2-type (immunoglobulin-like) domains. Database links: Entrez Gene: 4607 Human Entrez Gene: 17868 Mouse Entrez Gene: 295929 Rat Omim: 600958 Human SwissProt: Q14896 Human SwissProt: O70468 Mouse SwissProt: P56741 Rat Unigene: 524906 Human Unigene: 10728 Mouse Unigene: 162668 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or 心脏肌球蛋白结合蛋白抗体diagnostic applications. Involvement in disease: Defects in MYBPC3 are the cause of cardiomyopathy familial hypertrophic type 4 (CMH4). 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.
产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 （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.
研究领域：心血管 **学
心脏肌球蛋白结合蛋白抗体储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid