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Rabbit Anti-CRTAP/BF488 Conjugated antibody (bs-12949R-BF488)
訂購熱線:400-901-9800
訂購郵箱:sales@xucheq.com
訂購QQ:  400-901-9800
技術支持:techsupport@xucheq.com
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-12949R-BF488
英文名稱1 Rabbit Anti-CRTAP/BF488 Conjugated antibody
中文名稱 BF488標記的軟骨相關蛋白CRTAP抗體
別    名 Cartilage associated protein; LEPREL3; leprecan-like 3; CASP; CRTAP_HUMAN.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  信號轉(zhuǎn)導  細胞外基質(zhì)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Horse, Rabbit, )
產(chǎn)品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 44kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CRTAP
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 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 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.

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.
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