软骨寡聚基质蛋白抗体

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英文名称  Anti-COMP/EPD1/THBS5

中文名称  软骨寡聚基质蛋白抗体

别    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.

浓    度  1mg/1ml

规 格  0.1ml/100μg  0.2ml/200μg  

软骨寡聚基质蛋白抗体概述：

B淋巴细胞在抗原的刺激下，能够分化、增殖形成具有针对这种抗原分泌特异性抗体的能力。B细胞的这种能力和量是有限的，不可能持续分化增殖下去，因此产生免疫球蛋白的能力也是极其微小的。将这种B细胞与非分泌型的骨髓瘤细胞融合形成杂交瘤细胞，再进一步克隆化，这种克隆化的杂交瘤细胞是既具有瘤细胞的无限分裂的能力，又具有产生特异性抗体的B淋巴细胞的能力。将这种克隆化的杂交瘤细胞进行培养或注入小鼠腹水内即可获得大量的高效、单一的特异性抗体。这种技术即称为单克隆抗体技术。

抗体来源  Rabbit  

克隆类型  polyclonal

交叉反应  Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Daniorerio  

产品类型  一抗    

研究领域  细胞生物 免疫学 信号转导 转录调节因子 细胞粘附分子  

蛋白分子量  predicted molecular weight: 83kDa

性    状  Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human COMP

亚    型  IgG

纯化方法  affinity purified by Protein A

储 存 液  0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide

产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=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.  

保存条件  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.

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

产品介绍 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.

软骨寡聚基质蛋白抗体Similarity : Belongs to the thrombospondin family.

Contains 4 EGF-like domains.

Contains 1 TSP C-terminal (TSPC) domain.

Contains 8 TSP type-3 repeats.

Database links : UniProtKB/Swiss-Prot: P49747.2