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COMP Rabbit pAb (bs-10286R)  
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產(chǎn)品編號(hào) bs-10286R
英文名稱 COMP Rabbit pAb
中文名稱 軟骨寡聚基質(zhì)蛋白抗體
別    名 Cartilage oligomeric matrix protein; Cartilage oligomeric matrix protein precursor; EDM 1; EDM1; EPD 1; EPD1; Epiphyseal dysplasia 1; Epiphyseal dysplasia 1 multiple; Epiphyseal dysplasia multiple 1; MED; MGC13181; MGC149768; PSACH; Pseudoachondroplasia; THBS 5; THBS5; Thrombospondin 5; Thrombospondin5.  
Specific References  (1)     |     bs-10286R has been referenced in 1 publications.
[IF=1.879] Guangdi Li. et al. The relationship between abnormal Core binding factor-β expression in human cartilage and osteoarthritis. Bmc Musculoskel Dis. 2021 Dec;22(1):1-13  WB ;  Human.  
研究領(lǐng)域 細(xì)胞生物  免疫學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  轉(zhuǎn)錄調(diào)節(jié)因子  細(xì)胞粘附分子  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
克 隆 號(hào)
交叉反應(yīng) Human (predicted: Mouse,Rat,Pig,Sheep,Cow,Dog,Horse)
產(chǎn)品應(yīng)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 83 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞外基質(zhì) 分泌型蛋白 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human COMP: 221-320/757 
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(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 The protein encoded by this gene is a noncollagenous extracellular matrix (ECM) protein. It consists of five identical glycoprotein subunits, each with EGF-like and calcium-binding (thrombospondin-like) domains. Oligomerization results from formation of a five-stranded coiled coil and disulfides. Binding to other ECM proteins such as collagen appears to depend on divalent cations. Mutations can cause the osteochondrodysplasias pseudochondroplasia (PSACH) and multiple epiphyseal dysplasia (MED). [provided by RefSeq, Jul 2008].

Function:
May play a role in the structural integrity of cartilage via its interaction with other extracellular matrix proteins such as the collagens and fibronectin. Can mediate the interaction of chondrocytes with the cartilage extracellular matrix through interaction with cell surface integrin receptors. Could play a role in the pathogenesis of osteoarthritis. Potent suppressor of apoptosis in both primary chondrocytes and transformed cells. Suppresses apoptosis by blocking the activation of caspase-3 and by inducing the IAP family of survival proteins (BIRC3, BIRC2, BIRC5 and XIAP). Essential for maintaining a vascular smooth muscle cells (VSMCs) contractile/differentiated phenotype under physiological and pathological stimuli. Maintains this phenotype of VSMCs by interacting with ITGA7.

Subunit:
Pentamer; disulfide-linked. Exists in a more compact conformation in the presence of calcium and shows a more extended conformation in the absence of calcium. Interacts with ITGB3, ITGA5 and FN1. Binding to FN1 requires the presence of divalent cations (Ca(2+), Mg(2+) or Mn(2+)). The greatest amount of binding is seen in the presence of Mn(2+). Interacts with MATN1, MATN3, MATN4 and ACAN. Binds heparin, heparan sulfate and chondroitin sulfate. EDTA dimishes significantly its binding to ACAN and abolishes its binding to MATN3, MATN4 and chondroitin sulfate. Interacts with collagen I, II and IX, and interaction with these collagens is dependent on the presence of zinc ions. Interacts with ADAMTS12. Interacts with ITGA7.

Subcellular Location:
Secreted, extracellular space, extracellular matrix.

Tissue Specificity:
Abundantly expressed in the chondrocyte extracellular matrix, and is also found in bone, tendon, ligament and synovium and blood vessels. Increased amounts are produced during late stages of osteoarthritis in the area adjacent to the main defect.

DISEASE:
Defects in COMP are the cause of multiple epiphyseal dysplasia type 1 (EDM1) [MIM:132400]. EDM is a generalized skeletal dysplasia associated with significant morbidity. Joint pain, joint deformity, waddling gait, and short stature are the main clinical signs and symptoms. EDM is broadly categorized into the more severe Fairbank and the milder Ribbing types.
Defects in COMP are the cause of pseudoachondroplasia (PSACH) [MIM:177170]. PSAC is a dominantly inherited chondrodysplasia characterized by short stature and early-onset osteoarthrosis. PSACH is more severe than EDM1 and is recognized in early childhood.

Similarity:
Belongs to the thrombospondin family.
Contains 4 EGF-like domains.
Contains 1 TSP C-terminal (TSPC) domain.
Contains 8 TSP type-3 repeats.

SWISS:
P49747

Gene ID:
1311

Database links:

Entrez Gene: 1311 Human

Entrez Gene: 12845 Mouse

Entrez Gene: 25304 Rat

Omim: 600310 Human

SwissProt: P49747 Human

SwissProt: Q9R0G6 Mouse

SwissProt: P35444 Rat

Unigene: 1584 Human

Unigene: 45071 Mouse

Unigene: 10343 Rat



產(chǎn)品圖片
Protein: Raji lysate; Primary: rabbit Anti-COMP (bs-10286R) at 1:300; Secondary: HRP conjugated Goat-Anti-rabbit IgG(bs-0295G-HRP) at 1: 5000; Predicted band size: 83 kD Observed band size: 77 kD
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