软骨相关蛋白颁搁罢础笔抗体
规格：1尘驳/1尘濒
英文名： CRTAP
别名： Cartilage associated protein; LEPREL3; leprecan-like 3; CASP; CRTAP_HUMAN.
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：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human CRTAP
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Horse, Rabbit,
细胞定位：分泌型蛋白
软骨相关蛋白颁搁罢础笔抗体产物介绍：background: 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. Function: CRTAP is软骨相关蛋白颁搁罢础笔抗体 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. Subcellular Location: Secreted, extracellular space, extracellular matrix (By similarity). Tissue Specificity: Found in articular 软骨相关蛋白颁搁罢础笔抗体chondrocytes. Expressed in a variety of tissues. DISEASE: Defects in CRTAP are the cause of osteogenesis imperfecta type 7 (OI7) [MIM:610682]. A connective tissue disorder characterized by short stature, short humeri and femora, coxa vara, white sclera, and the absence of dentinogenesis imperfecta. Multiple fractures are present at birth, and patients manifest moderate-severe bone fragility. Death may occurr in the perinatal period due to secondary respiratory insufficiency. Similarity: Belongs to the leprecan family. Database links: UniProtKB/Swiss-Prot: O75718.1 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
软骨相关蛋白颁搁罢础笔抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=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：细胞生物 信号转导 细胞外基质
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid