Kielin cysteine-rich BMP regulator (KCP)

Kielin cysteine-rich BMP regulator (KCP) is a large, secreted cysteine-rich protein that modulates bone morphogenetic protein (BMP) and transforming growth factor beta (TGF-β) signaling pathways in the extracellular space[1][4]. KCP enhances BMP signaling, notably facilitating BMP7 binding to its receptor, which promotes osteoblast differentiation and mineralization and exerts protective effects in renal epithelial cells[3][5]. In contrast, KCP inhibits TGF-β and activin-mediated profibrotic signaling by sequestering these ligands, thus reducing their interaction with respective receptors[1][4]. Animal studies indicate that overexpression of KCP in the kidney attenuates renal fibrosis and improves recovery following acute kidney injury[1]. Variants in KCP have been linked to hereditary hemorrhagic telangiectasia and intellectual disability in some genetic studies[4]. KCP is under active investigation as a potential biotherapeutic modulator for fibrotic and bone diseases, though no drugs directly targeting KCP are currently approved.

Other names

Kielin/chordin-like proteinCRIM2KCP1CRIM-2KCP-1FLJ33365NET67Cysteine-rich BMP regulator 2Cysteine-rich motor neuron 2 proteinKielin/chordin-like protein 1

Enhances BMP signaling by increasing ligand-receptor interaction (promotes BMP7/BMP receptor 1A binding); Inhibits TGF-β and Activin signaling by sequestering ligands and blocking ligand-receptor interactions