CRTAP Antibody Blocking Peptide(bs-12949P)-500ug

CRTAP Antibody Blocking Peptid

e(bs-12949P)-500ug
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  • ¥880
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  • bs-12949P
  • 2025年10月16日
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      500ug

    产品编号bs-12949P
    英文名称CRTAP Antibody Blocking Peptide
    中文名称软骨相关蛋白CRTAP封闭多肽
    英文别名Cartilage associated protein; LEPREL3; leprecan-like 3; CASP; CRTAP_HUMAN.
    纯化方法HPLC
    研究领域

    Signal Transduction > Cytoskeleton / ECM > Extracellular Matrix > ECM Proteins > Other ECM Proteins

    Signal Transduction > Cytoskeleton / ECM > Extracellular Matrix > Structures > Bone

    亚细胞定位Secreted, extracellular space, extracellular matrix (By similarity).
    组织特异性Found in articular chondrocytes. Expressed in a variety of tissues.
    相似性Belongs to the leprecan family.
    功能CRTAP is found in articular chondrocytes and is expressed in a variety of other tissues. Defects in CRTAP are the cause of osteogenesis imperfecta type 7 (OI-7). OI is a connective tissue disorder characterized by bone fragility and low bone mass. OI-7 is an autosomal recessive form of OI.
    保存条件Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事项This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    背景资料CRTAP is a secreted protein localizing to the extracellular space that plays a role in collagen post-translational modifications, extracellular fibril assembly and intracellular trafficking. CRTAP is widely expressed with predominant expression in articular chondrocytes. It contains a signal peptide and a tetratricopeptide-like helical domain and is essential for normal bone formation. In the endoplasmic reticulum (ER), CRTAP forms a complex with Gros1 and CyPB (cyclophilin B) and is required for the efficient 3-hydroxylation of target prolyl residues in Collagen Type I molecules, the major structural proteins of skin and bone. Mutations in the gene encoding CRTAP can lead to autosomal recessive osteogenesis imperfecta (OI) type 7 and type 2B. OI, also known as brittle bone disease, is characterized by bone fragility and susceptibility to fractures. OI type 7 is a mild form of this disorder, while OI type 2B is a neonatal lethal condition.

     

     

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