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货号: bs-14087R 基本售价: 1380.0 元 规格: 100ul
- 规格:100ul
- 价格:1380.00元
- 规格:200ul
- 价格:2200.00元
产品信息
- 产品编号
- bs-14087R
- 英文名称
- CSRP3
- 中文名称
- 富含半胱氨酸蛋白3抗体
- 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; Cysteine-rich protein 3; LIM domain only 4; LIM domain protein; LIM domain protein cardiac; LMO4; MGC14488; MGC61993; MLP; Muscle LIM protein; Muscle LIM protein.
- 规格价格
- 100ul/1380元购买 200ul/2200元购买 大包装/询价
- 说 明 书
- 100ul 200ul
- 研究领域
- 心血管 细胞生物 信号转导 表观遗传学
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Pig, Cow, Sheep,
- 产品应用
- ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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.
- 分 子 量
- 21kDa
- 细胞定位
- 细胞核 细胞浆
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human CSRP3:101-194/194
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- PubMed
- PubMed
- 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 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 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.
SWISS:
P50461
Gene ID:
8048
Database links:Entrez Gene: 8048Human
Entrez Gene: 540407Cow
Entrez Gene: 13009Mouse
Entrez Gene: 100337687Pig
Entrez Gene: 117505Rat
Omim: 600824Human
SwissProt: Q4U0T9Cow
SwissProt: P50461Human
SwissProt: P50462Mouse
SwissProt: P50463Rat
Unigene: 83577Human
Unigene: 17235Mouse
Unigene: 11345Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.