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软骨寡聚基质蛋白抗体Ys-10286R
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详细内容
产品编号Ys-10286R
英文名称COMP
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.
抗体来源Rabbit
克隆类型Polyclonal
交叉反应(predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, )
产品应用WB=1:500-2000 ELISA=1:5000-10000 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.
理论分子量83kDa
细胞定位细胞外基质 分泌型蛋白
性 状Liquid
浓 度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human COMP: 221-320/757
亚 型IgG
纯化方法affinity purified by Protein A
缓 冲 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMedPubMed
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.
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
热门标签:软骨寡聚基质蛋白抗体,抗体













