热休克蛋白-47抗体
规格：1mg/1ml
英文名： HSP47
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; Colla
分子量： 44kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human HSP47
交叉反应：Human, Mouse, Rat,
细胞定位：细胞浆
热休克蛋白-47抗体产品介绍：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 热休克蛋白-47抗体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热休克蛋白-47抗体 affecting the gene represented in this entry. Similarity: Belongs to the serpin family. Gene ID: 871 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 热休克蛋白-47抗体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**系统识别,诱发机体的保护性**应答,也可作为一种信号分子,在信号转导中发挥作用.
产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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.
研究领域：肿瘤 **学 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit