Anti-Hsp47 Rabbit Monoclonal Antibody(BM4791-50ul)

Anti-Hsp47 Rabbit Monoclonal A

ntibody(BM4791-50ul)
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  • ¥1180
  • BOSTER已认证
  • BM4791-50ul
  • 中国
  • 2025年07月11日
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    • 详细信息
    • 技术资料
    • 库存

      大量

    • 供应商

      广州威佳科技有限公司

    • 规格

      50ul

    产品概况

    货号 BM4791
    产品名称 Anti-SERPINH1 Antibody
    基因名 SERPINH1
    抗体来源 Rabbit
    克隆 Monoclonal
    抗体亚型 Rabbit
    分子量 46KD
    免疫原 A synthesized peptide derived from human Hsp47
    内容 500 ug/ml;Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
    纯化方式 Affinity-chromatography
    浓度 500 ug/ml
    产品形态 Liquid
    保存条件 12 months from date of receipt,-20℃ as supplied. 6 months 2 to 8℃ after reconstitution. Avoid repeated freezing and thawing.
    背景资料 Heat shock protein 47, also known as SERPINH1 or HSP47, is a serpin which serves as a human chaperone protein for collagen. 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. It has been found that HSP47 monitors the integrity of the triple helix of type I procollagen at the ER/cis-Golgi boundary and, when absent, the rate of transit from the ER to the Golgi is increased and the helical structure is compromised.
    研究类别 1. Christiansen, H. E., Schwarze, U., Pyott, S. M., AlSwaid, A., Al Balwi, M., Alrasheed, S., Pepin, M. G., Weis, M. A., Eyre, D. R., Byers, P. H. Homozygosity for a missense mutation in SERPINH1, which encodes the collagen chaperone protein HSP47, results in severe recessive osteogenesis imperfecta. Am. J. Hum. Genet. 86: 389-398, 2010.2. "Entrez Gene: SERPINH1 serpin peptidase inhibitor, clade H (heat shock protein 47), member 1, (collagen binding protein 1)"3. Dafforn T, Della M, Miller A (2001). "The molecular interactions of heat shock protein 47 (Hsp47) and their implications for collagen biosynthesis". J. Biol. Chem. 276(52): 49310–9.
    Uniprot ID SERPINH1: P50454

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    [list_product_images]Western blot analysis of Hsp47 expression in HeLa cell lysate.[/list_product_images]

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