Role of calcium in polycystic kidney disease: From signaling to pathology.
Ferrara, Italy. In World J Nephrol, Feb 2016
The PKD1 gene encodes for polycystin-1 (PC1), a large multi-functional membrane receptor protein able to regulate ion channel complexes, whereas polycystin-2 (PC2), encoded by the PKD2 gene, is an integral membrane protein that functions as a calcium-permeable cation channel, located mainly in the endoplasmic reticulum (ER).
The functions of TRPP2 in the vascular system.
Hefei, China. In Acta Pharmacol Sin, Jan 2016
TRPP2 (polycystin-2, PC2 or PKD2), encoded by the PKD2 gene, is a non-selective cation channel with a large single channel conductance and high Ca(2+) permeability.
FGF signalling regulates bone growth through autophagy.
Pozzuoli, Italy. In Nature, Jan 2016
Mice lacking the autophagy related gene 7 (Atg7) in chondrocytes experience endoplasmic reticulum storage of type II procollagen (PC2) and defective formation of the Col2 fibrillary network in the ECM.
Autosomal Dominant Polycystic Kidney Disease: A Path Forward.
Sydney, Australia. In Semin Nephrol, Nov 2015
Autosomal dominant polycystic kidney disease (ADPKD) is the commonest inherited cause of renal failure in adults, and is due to loss-of-function mutations in either the PKD1 or PKD2 genes, which encode polycystin-1 and polycystin-2, respectively.
Direct recording and molecular identification of the calcium channel of primary cilia.
Boston, United States. In Nature, 2014
The polycystin proteins (PC and PKD), identified in linkage studies of polycystic kidney disease, are candidate channels divided into two structural classes: 11-transmembrane proteins (PKD1, PKD1L1 and PKD1L2) remarkable for a large extracellular amino terminus of putative cell adhesion domains and a G-protein-coupled receptor proteolytic site, and the 6-transmembrane channel proteins (PKD2, PKD2L1 and PKD2L2; TRPPs).
The biology and therapeutic targeting of the proprotein convertases.
Montréal, Canada. In Nat Rev Drug Discov, 2012
Seven of these (proprotein convertase 1 (PC1), PC2, furin, PC4, PC5, paired basic amino acid cleaving enzyme 4 (PACE4) and PC7) activate cellular and pathogenic precursor proteins by cleavage at single or paired basic residues, whereas subtilisin kexin isozyme 1 (SKI-1) and proprotein convertase subtilisin kexin 9 (PCSK9) regulate cholesterol and/or lipid homeostasis via cleavage at non-basic residues or through induced degradation of receptors.