产品名称
COMP Rabbit Polyclonal Antibody
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
https://www.uniprot.org/uniprot/1311
Human Swissprot No.
P49747
Human Swissprot Link
https://www.uniprot.org/uniprotkb/P49747/entry
Mouse Gene Link
https://www.uniprot.org/uniprot/12845
Mouse Swissprot No.
Q9R0G6
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/Q9R0G6
Rat Gene Link
https://www.uniprot.org/uniprot/25304
Rat Swissprot Link
https://www.uniprot.org/uniprotkb/P35444
免疫原
Synthesized peptide derived from human COMP AA range: 628-678
特异性
This antibody detects endogenous levels of COMP at Human/Mouse/Rat
宿主
Polyclonal, Rabbit,IgG
背景介绍
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. Contraction or expansion of a 5 aa aspartate repeat and other mutations can cause pseudochondroplasia (PSACH) and multiple epiphyseal dysplasia (MED). [provided by RefSeq, Jul 2016],
组织表达
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.
细胞定位
Secreted, extracellular space, extracellular matrix .
功能
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.,disease: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.,similarity:Contains 1 TSP C-terminal (TSPC) domain.,similarity:Contains 4 EGF-like domains.,similarity:Contains 8 TSP type-3 repeats.,subunit:Pentamer; disulfide-linked.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
