Disease relevance of Crim2

• Mice homozygous for a KCP null allele are hypersensitive to developing renal interstitial fibrosis, a disease stimulated by TGF-beta but inhibited by BMP7 [1].

High impact information on Crim2

• Consistent with this inhibitory effect, primary renal epithelial cells from KCP mutant cells are hypersensitive to TGF-beta and exhibit increased apoptosis, dissociation of cadherin-based cell junctions, and expression of smooth muscle actin [1].
• Furthermore, KCP null animals show elevated levels of phosphorylated Smad2 after renal injury [1].
• The ability to enhance BMP signaling while suppressing TGF-beta activation indicates a critical role for KCP in modulating the responses between these anti- and profibrotic cytokines in the initiation and progression of renal interstitial fibrosis [1].
• The cysteine-rich domain protein KCP is a suppressor of transforming growth factor beta/activin signaling in renal epithelia [1].

Biological context of Crim2

• KCP is an enhancer of BMP signaling in vertebrates and interacts with BMPs and the BMP type I receptor to promote receptor-ligand interactions [1].

References