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- 详细信息
- 技术资料
- 规格:
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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CRTAP Antibody Blocking Peptide(bs-12949P)-500ug
¥880





