CILP O75339

软骨中间层蛋白 1 (CILP-1) (软骨中间层蛋白) [裂解为：软骨中间层蛋白 1 C1；软骨中间层蛋白 1 C2]

功能描述

可能在软骨支架中发挥作用。可能通过拮抗TGF-beta1 (TGFB1)和IGF1的功能起作用。具有抑制IGF1诱导的增殖和硫酸化蛋白多糖合成的能力，并抑制配体诱导的IGF1R自身磷酸化。可能通过与TGFB1相互作用抑制TGFB1介导的软骨基质基因诱导。过表达可能导致软骨细胞生长和基质修复受损，并间接促进衰老和骨关节炎软骨中无机焦磷酸盐(PPi)的过饱和。 {ECO:0000269|PubMed:12746903, ECO:0000269|PubMed:15864306}。

组织特异性

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.

亚细胞定位

Secreted, extracellular space, extracellular matrix