CILP

Description

The CILP (cartilage intermediate layer protein) is a protein-coding gene located on chromosome 15.

Cartilage intermediate layer protein 1 is a protein encoded by the CILP gene in humans. Changes in the composition of the cartilage extracellular matrix are significant in joint diseases like osteoarthritis. The synthesis of CILP, isolated and purified from human articular cartilage, increases in early osteoarthritis cartilage. The C-terminal 460 amino acids of the protein exhibit 90% similarity to the pig ectonucleotide pyrophosphohydrolase NTPPHase; this region is preceded by a furin protease consensus cleavage site. Therefore, the CILP gene is believed to encode a protein precursor for two distinct proteins: CILP and a homolog of NTPPHase.

CILP plays a role in cartilage scaffolding by potentially antagonizing the functions of TGF-beta1 (TGFB1) and IGF1. It can suppress IGF1-induced proliferation and sulfated proteoglycan synthesis, and inhibit ligand-induced IGF1R autophosphorylation. CILP may also inhibit TGFB1-mediated induction of cartilage matrix genes by interacting with TGFB1. Overexpression of CILP could impair chondrocyte growth and matrix repair, indirectly promoting inorganic pyrophosphate (PPi) supersaturation in aging and osteoarthritis cartilage.

CILP is also known as CILP-1, CILP1, HsT18872.

Associated Diseases

• nail-patella syndrome
• breast cancer
• familial generalized lentiginosis
• linear atrophoderma of Moulin
• Dowling-Degos disease 1
• acroleukopathy, symmetric
• familial progressive hyperpigmentation
• dyschromatosis symmetrica hereditaria
• hyperpigmentation with or without hypopigmentation, familial progressive
• tufted angioma
• colorectal cancer
• Duchenne muscular dystrophy