Cartilage intermediate layer protein (CILP), an extracellular matrix protein, shows abundant expression in cartilagenous tissues. CILP is expressed as two isoforms, CILP and CILP-2, which are differentially expressed by chondrocytes and induced by TGF∫1. This induction is mediated by Smad3 through direct interactions with cis-elements in the CILP promoter region. TGF∫ also induces elevated chondrocyte extracellular inorganic pyrophosphate (PPi), which promotes the deposition of calcium pyrophosphate dihydrate crystals. The CILP isoforms have been implicated in common musculoskeletal disorders, including osteoarthritis, rheumatoid arthritis and lumbar disc disease.
Function:
May play a role in cartilage scaffolding.
Subcellular Location:
Secreted > extracellular space > extracellular matrix.
Tissue Specificity:
Expressed in articular chondrocytes but not in knee meniscal cartilage cells.
Post-translational modifications:
May be cleaved into 2 chains possibly by a furin-like protease upon or preceding secretion.
Similarity:
Contains 1 Ig-like C2-type (immunoglobulin-like) domain.
Contains 1 TSP type-1 domain.
SWISS:
Q8IUL8
Gene ID:
148113
Database links:
Entrez Gene: 148113 Human
Omim: 612419 Human
SwissProt: Q8IUL8 Human
Unigene: 279574 Human
|
|
