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Rabbit Anti-SerpinB6/PE Conjugated antibody (bs-19659R-PE)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-19659R-PE
英文名称1 Rabbit Anti-SerpinB6/PE Conjugated antibody
中文名称 PE标记的丝氨酸蛋白酶抑制剂B6抗体
别    名 CAP; Cytoplasmic antiproteinase; Peptidase inhibitor 6; PI-6; PI6; Placental thrombin inhibitor; Protease inhibitor 6 (placental thrombin inhibitor); PTI; Serpin B6; SERPINB6; SPB6_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  免疫学  泛素  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Monkey, )
产品应用 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 43kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SerpinB6
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 protein encoded by this gene is a member of the serpin (serine proteinase inhibitor) superfamily, and ovalbumin(ov)-serpin subfamily. It was originally discovered as a placental thrombin inhibitor. The mouse homolog was found to be expressed in the hair cells of the inner ear. Mutations in this gene are associated with nonsyndromic progressive hearing loss, suggesting that this serpin plays an important role in the inner ear in the protection against leakage of lysosomal content during stress, and that loss of this protection results in cell death and sensorineural hearing loss. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Sep 2010]

Function:
May be involved in the regulation of serine proteinases present in the brain or extravasated from the blood (By similarity). Inhibitor of cathepsin G, kallikrein-8 and thrombin. May play an important role in the inner ear in the protection against leakage of lysosomal content during stress and loss of this protection results in cell death and sensorineural hearing loss.

Subcellular Location:
Cytoplasm.

Tissue Specificity:
Expressed in keratinocytes (at protein level). Highest levels in skeletal muscle. Also found in placenta, cardiac muscle, lung, liver, kidney and pancreas. Expressed in the inner ear hair cells. Expressed abundantly by normal mast cells in different tissues and by mast cells in mastocytoma lesions.

DISEASE:
Defects in SERPINB6 are the cause of deafness autosomal recessive type 91 (DFNB91) [MIM:613453]. It is a form of non-syndromic deafness characterized by progressive and age-dependent sensorineural hearing loss. Vestibular function is normal.

Similarity:
Belongs to the serpin family. Ov-serpin subfamily.

Database links:

Entrez Gene: 5269 Human

Entrez Gene: 20719 Mouse

Omim: 173321 Human

SwissProt: P35237 Human

SwissProt: Q60854 Mouse

Unigene: 519523 Human

Unigene: 252210 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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