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Rabbit Anti-Cystatin A/BF488 Conjugated antibody (bs-5114R-BF488)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-5114R-BF488
英文名称 Rabbit Anti-Cystatin A/BF488 Conjugated antibody
中文名称 BF488标记的胱抑素A/半胱氨酸蛋白酶抑制剂A抗体
别    名 CSTA; CSTA protein; Cystatin A; Cystatin AS; Cystatin-A; Cystatin-AS; CystatinA; CYTA_HUMAN; Stefin A; Stefin A1; Stefin-A; StefinA; STF 1; STF1; STFA.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  细胞生物  免疫学  信号转导  细胞凋亡  转录调节因子  细胞类型标志物  细胞骨架  肿瘤细胞生物标志物  细胞外基质  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human,  (predicted: Dog, Cow, )
产品应用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 11kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Cystatin A
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.
产品介绍 background:
The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins, and kininogens. This gene encodes a stefin that functions as a cysteine protease inhibitor, forming tight complexes with papain and the cathepsins B, H, and L. The protein is one of the precursor proteins of cornified cell envelope in keratinocytes and plays a role in epidermal development and maintenance. Stefins have been proposed as prognostic and diagnostic tools for cancer. [provided by RefSeq, Jul 2008]

Function:
This is an intracellular thiol proteinase inhibitor. Has an important role in desmosome-mediated cell-cell adhesion in the lower levels of the epidermis.

Subcellular Location:
Cytoplasm.

Tissue Specificity:
Expressed in the skin throughout the epidermis.

DISEASE:
Defects in CSTA are the cause of ichthyosis exfoliative autosomal recessive ichthyosis bullosa of Siemens-like (AREI) [MIM:607936]. A form of congenital exfoliative ichthyosis, sharing some features with ichthyosis bullosa of Siemens and annular epidermolytic ichthyosis. AREI presents shortly after birth as dry, scaly skin over most of the body with coarse peeling of non-erythematous skin on the palms and soles, which is exacerbated by excessive moisture and minor trauma. Electron microscopy analysis of skin biopsies, reveals mostly normal-appearing upper layers of the epidermis, but prominent intercellular edema of the basal and suprabasal cell layers with aggregates of tonofilaments in the basal keratinocytes.

Similarity:
Belongs to the cystatin family.

Database links:

Entrez Gene: 1475 Human

Entrez Gene: 209294 Mouse

Omim: 184600 Human

SwissProt: P01040 Human

SwissProt: P56567 Mouse

Unigene: 518198 Human

Unigene: 300592 Mouse



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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