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CRTAP Rabbit pAb (bs-12949R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-12949R
英文名称 CRTAP Rabbit pAb
中文名称 软骨相关蛋白CRTAP抗体
别    名 Cartilage associated protein; LEPREL3; leprecan-like 3; CASP; CRTAP_HUMAN.  
研究领域 细胞生物  信号转导  细胞外基质  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human (predicted: Mouse,Rat,Rabbit,Pig,Chicken,Dog,Horse)
产品应用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 44 kDa
检测分子量
细胞定位 分泌型蛋白 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CRTAP: 201-300/401 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store 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.
PubMed PubMed
产品介绍 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 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 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.

SWISS:
O75718

Gene ID:
10491

Database links:

Entrez Gene: 10491 Human

Entrez Gene: 56693 Mouse

Entrez Gene: 363158 Rat

Omim: 605497 Human

SwissProt: O75718 Human

SwissProt: Q9CYD3 Mouse



产品图片
Sample: U251(Human) Cell Lysate at 30 ug A549(Human) Cell Lysate at 30 ug Primary: Anti- CRTAP (bs-12949R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 44 kD Observed band size: 44 kD
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