功能： caution:Was originally thought to constitute the ATP pyrophosphatase enzyme (NTPPH). However, it was later shown (PubMed:12746903 and PubMed:15864306) that it is not the case.,disease:Antibodies against CILP are detected in patients with early-stage knee osteoarthritis (OA) and rheumatoid arthritis (RA). Autoantibodies against the C1 and C2 chains are detected in 10 out of 136 and 17 out of 136 patient with OA, respectively.,disease:Defects in CILP may be a cause of susceptibility to lumbar disk disease (LDD). LDD is caused by degeneration of intervertebral disk of the lumbar spine, and its associated disk degeneration and concomitant disk herniation are a primary cause of low back pain and unilateral leg pain (sciatica).,function:Probably plays a role in cartilage scaffolding. May act by antagonizing TGF-beta1 (TGFB1) and IGF1 functions. Has the ability to suppress IGF1-induced proliferation and sulfated proteoglycan synthesis, and inhibits ligand-induced IGF1R autophosphorylation. May inhibit TGFB1-mediated induction of cartilage matrix genes via its interaction with TGFB1. Overexpression may lead to impair chondrocyte growth and matrix repair and indirectly promote inorganic pyrophosphate (PPi) supersaturation in aging and osteoarthritis cartilage.,PTM:Cleaved into 2 chains possibly by a furin-like protease upon or preceding secretion.,similarity:Contains 1 Ig-like C2-type (immunoglobulin-like) domain.,similarity:Contains 1 TSP type-1 domain.,subunit:Monomer. Interacts with TGFB1.,tissue specificity:Specifically expressed in cartilage. Localizes in the intermediates layer of articular cartilage but neither in the superficial nor in the deepest regions. Specifically and highly expressed in intervertebral disk tissue. Expression increases with aging in hip articular cartilage. Overexpressed in articular hyaline cartilage from patients with calcium pyrophosphate dihydrate crystal deposition disease (CPPD). Expression in intervertebral disk tissue from individuals with LDD increases as disk degeneration progressed.,
组织表达： Specifically expressed in cartilage. Localizes in the intermediates layer of articular cartilage but neither in the superficial nor in the deepest regions. Specifically and highly expressed in intervertebral disk tissue. Expression increases with aging in hip articular cartilage. Overexpressed in articular hyaline cartilage from patients with calcium pyrophosphate dihydrate crystal deposition disease (CPPD). Expression in intervertebral disk tissue from individuals with lumbar disk disease increases as disk degeneration progresses.
科研货号： PLA007799
CILP1 Polyclonal Antibody
Catalog NoPLA007799
Product information
基因名称： CILP UNQ602/PRO1188
蛋白名称： Cartilage intermediate layer protein 1 (CILP-1) (Cartilage intermediate-layer protein) [Cleaved into: Cartilage intermediate layer protein 1 C1; Cartilage intermediate layer protein 1 C2]
功能： caution:Was originally thought to constitute the ATP pyrophosphatase enzyme (NTPPH). However, it was later shown (PubMed:12746903 and PubMed:15864306) that it is not the case.,disease:Antibodies against CILP are detected in patients with early-stage knee osteoarthritis (OA) and rheumatoid arthritis (RA). Autoantibodies against the C1 and C2 chains are detected in 10 out of 136 and 17 out of 136 patient with OA, respectively.,disease:Defects in CILP may be a cause of susceptibility to lumbar disk disease (LDD). LDD is caused by degeneration of intervertebral disk of the lumbar spine, and its associated disk degeneration and concomitant disk herniation are a primary cause of low back pain and unilateral leg pain (sciatica).,function:Probably plays a role in cartilage scaffolding. May act by antagonizing TGF-beta1 (TGFB1) and IGF1 functions. Has the ability to suppress IGF1-induced proliferation and sulfated proteoglycan synthesis, and inhibits ligand-induced IGF1R autophosphorylation. May inhibit TGFB1-mediated induction of cartilage matrix genes via its interaction with TGFB1. Overexpression may lead to impair chondrocyte growth and matrix repair and indirectly promote inorganic pyrophosphate (PPi) supersaturation in aging and osteoarthritis cartilage.,PTM:Cleaved into 2 chains possibly by a furin-like protease upon or preceding secretion.,similarity:Contains 1 Ig-like C2-type (immunoglobulin-like) domain.,similarity:Contains 1 TSP type-1 domain.,subunit:Monomer. Interacts with TGFB1.,tissue specificity:Specifically expressed in cartilage. Localizes in the intermediates layer of articular cartilage but neither in the superficial nor in the deepest regions. Specifically and highly expressed in intervertebral disk tissue. Expression increases with aging in hip articular cartilage. Overexpressed in articular hyaline cartilage from patients with calcium pyrophosphate dihydrate crystal deposition disease (CPPD). Expression in intervertebral disk tissue from individuals with LDD increases as disk degeneration progressed.,
组织表达： Specifically expressed in cartilage. Localizes in the intermediates layer of articular cartilage but neither in the superficial nor in the deepest regions. Specifically and highly expressed in intervertebral disk tissue. Expression increases with aging in hip articular cartilage. Overexpressed in articular hyaline cartilage from patients with calcium pyrophosphate dihydrate crystal deposition disease (CPPD). Expression in intervertebral disk tissue from individuals with lumbar disk disease increases as disk degeneration progresses.
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