富含半胱氨酸蛋白3抗体
规格:1mg/1ml
英文名: CSRP3
别名: cardiac; Cardiac LIM protein; CLP; CMD1M; CMH12; CRP3; CSRP 3; Csrp3; CSRP3 protein.; CSRP3_HUMAN; Cysteine and glycine rich protein 3; Cysteine and glycine-rich protein 3; Cysteine rich protein 3; Cy
分子量: 21kDa
储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型:Polyclonal
亚型:IgG
纯化方法:affinity purified by Protein A
**原:KLH conjugated synthetic peptide derived from human CSRP3
交叉反应:Human, Mouse, Rat, Chicken, Pig, Cow, Sheep,
细胞定位:细胞核 细胞浆
富含半胱氨酸蛋白3抗体产品介绍:background: This gene encodes a member of the CSRP family of LIM domain proteins, which may be involved in regulatory processes important for development and cellular differentiation. The LIM/double zinc-finger motif found in this protein is found in a group of proteins with critical functions in gene regulation, cell growth, and somatic differentiation. Mutations in this gene are thought to cause heritable forms of hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) in humans. Alternatively spliced transcript variants with different 5' UTR, but encoding the same protein, have been found for this gene. [provided by RefSeq, Jul 2008] Function: Positive regulator of myogenesis. Plays a crucial and specific role in the organization of cytosolic structures in cardiomyocytes. Could play a role in mechanical stretch sensing. May be a scaffold protein that promotes the assembly of interacting proteins at Z-line structures. It is essential for calcineurin anchorage to富含半胱氨酸蛋白3抗体 the Z line. Required for stress-induced calcineurin-NFAT activation. Subcellular Location: Nucleus. Cytoplasm. Cytoplasm; cytoskeleton. Cytoplasm; myofibril; sarcomere; Z line. Mainly cytoplasmic (By similarity). In the nucleus it associates with the actin cytoskeleton (Potential). In the Z line, found associated with GLRX3. Tissue Specificity: Cardiac and slow-twitch skeletal muscles. DISEASE: Defects in CSRP3 are the cause of cardiomyopathy dilated type 1M (CMD1M) [MIM:607482]. 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 CSRP3 are the cause of cardiomyopathy familial hypertrophic type 12 (CMH12) [MIM:612124]. Familial hypertrophic cardiomyopathy is a hereditary heart disorder characterized by 富含半胱氨酸蛋白3抗体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. Similarity: Contains 2 LIM zinc-binding domains. Gene ID: 8048 Database links: Entrez Gene: 8048 Human Entrez Gene: 540407 Cow Entrez Gene: 13009 Mouse Entrez Gene: 100337687 Pig Entrez Gene: 117505 Rat Omim: 600824 Human SwissProt: Q4U0T9 Cow SwissProt: P50461 Human SwissProt: P50462 Mouse SwissProt: P50463 Rat Unigene: 83577 Human Unigene: 17235 Mouse Unigene: 11345 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
富含半胱氨酸蛋白3抗体产品应用:WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=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.
研究领域:心血管 细胞生物 信号转导 表观遗传学
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid