软骨寡聚基质蛋白抗体

软骨寡聚基质蛋白抗体

规格：1mg/1ml

英文名： COMP

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;

分子量： 83kDa

储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce

克隆类型：Polyclonal

亚型：IgG

纯化方法：affinity purified by Protein A

**原：KLH conjugated synthetic peptide derived from human COMP

交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep,

细胞定位：细胞外基质 分泌型蛋白

软骨寡聚基质蛋白抗体产品介绍：background: 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. 软骨寡聚基质蛋白抗体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 软骨寡聚基质蛋白抗体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. 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 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

软骨寡聚基质蛋白抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=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.

研究领域：细胞生物  **学  信号转导  转录调节因子  细胞粘附分子  

储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

来源： Rabbit

外观： Lyophilized or Liquid

产品留言

标题

联系人

联系电话

内容

验证码

点击换一张

注：1.可以使用快捷键Alt+S或Ctrl+Enter发送信息!
2.如有必要,请您留下您的详细联系方式!