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Rabbit Anti-CSRP3/RBITC Conjugated antibody (bs-14087R-RBITC)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號 bs-14087R-RBITC
英文名稱1 Rabbit Anti-CSRP3/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)標(biāo)記的富含半胱氨酸蛋白3抗體
別    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.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 心血管  細(xì)胞生物  信號轉(zhuǎn)導(dǎo)  表觀遺傳學(xué)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Chicken, Pig, Cow, Sheep, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 21kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CSRP3
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 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.

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.
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