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CRTAP Rabbit pAb (bs-12949R)  
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產品編號 bs-12949R
英文名稱 CRTAP Rabbit pAb
中文名稱 軟骨相關蛋白CRTAP抗體
別    名 Cartilage associated protein; LEPREL3; leprecan-like 3; CASP; CRTAP_HUMAN.  
研究領域 細胞生物  信號轉導  細胞外基質  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Human (predicted: Mouse,Rat,Rabbit,Pig,Chicken,Dog,Horse)
產品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 44 kDa
檢測分子量
細胞定位 分泌型蛋白 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CRTAP: 201-300/401 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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



產品圖片
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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