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Rabbit Anti-HSP47/HRP Conjugated antibody (bs-1538R-HRP)
~~~促销,代码KXJ230206-6~~~
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-1538R-HRP
英文名称 Anti-HSP47/HRP
中文名称 辣根过氧化物酶标记的热休克蛋白-47抗体
别    名 47 kDa heat shock protein; 47 kDa heat shock protein precursor; Arsenic transactivated protein 3; AsTP 3; AsTP3; CBP 1; CBP 2; CBP1; CBP2; Collagen binding protein 1; Collagen binding protein 2; Collagen binding protein; Colligen; Colligin 1; Colligin 2; Colligin; gp46; Heat shock protein 47; HGNC 1547; Hsp 47; J6; PIG 14; PIG14; PPROM; Proliferation inducing gene 14; Proliferation inducing gene 14 protein; RA A47; Rheumatoid arthritis antigen A 47; Rheumatoid arthritis related antigen RA A47; Serine or cysteine proteinase inhibitor clade H member 1; Serine or cysteine proteinase inhibitor clade H member 2; Serpin H1; Serpin peptidase inhibitor clade H member 1; SERPINH1; SERPINH2; SERPH_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  免疫学  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human,  (predicted: Mouse, Rat, )
产品应用 WB=1:500-2000 ELISA=1:100-1000 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 44kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HSP47
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:
This protein is a member of the serpin superfamily of serine proteinase inhibitors. Its expression is induced by heat shock. The protein localizes to the endoplasmic reticulum lumen and binds collagen; thus it is thought to be a molecular chaperone involved in the maturation of collagen molecules. Autoantibodies to this protein have been found in patients with rheumatoid arthritis.

Function:
Binds specifically to collagen. Could be involved as a chaperone in the biosynthetic pathway of collagen.

Subcellular Location:
Endoplasmic reticulum lumen.

DISEASE:
Osteogenesis imperfecta 10 (OI10) [MIM:613848]: A form of osteogenesis imperfecta, a connective tissue disorder characterized by low bone mass, bone fragility and susceptibility to fractures after minimal trauma. Disease severity ranges from very mild forms without fractures to intrauterine fractures and perinatal lethality. Extraskeletal manifestations, which affect a variable number of patients, are dentinogenesis imperfecta, hearing loss, and blue sclerae. OI10 is an autosomal recessive form characterized by multiple bone deformities and fractures, generalized osteopenia, dentinogenesis imperfecta, and blue sclerae. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the serpin family.

Database links:

Entrez Gene: 510850 Cow

Entrez Gene: 871 Human

Entrez Gene: 12406 Mouse

Entrez Gene: 29345 Rat

Omim: 600943 Human

SwissProt: Q2KJH6 Cow

SwissProt: P50454 Human

SwissProt: P19324 Mouse

SwissProt: P29457 Rat

Unigene: 596449 Human

Unigene: 22708 Mouse

Unigene: 444951 Mouse

Unigene: 98199 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

信号传导(Signaling Intermediates)
HSP47是一类进化上高度保守的蛋白质家族.生理状态时协助多肽或蛋白质的正确转位、折叠和装配,起"分子伴侣"的作用;在应激状态下,HSP47过表达或异位表达,作为一种自身抗原被免疫系统识别,诱发机体的保护性免疫应答,也可作为一种信号分子,在信号转导中发挥作用.
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