Phosphorylation, protein kinases and ADPKD

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

卷:1812

Review

Li, Xiaohong¹ 

[1] NY State Inst Basic Res Dev Disabil, Dept Neurochem, New York, NY 10314 USA

关键词: Polycystic kidney disease;    PRKX;    cAMP-dependent protein kinase;    Phosphorylation;    Polycystin-1;    Polycystin-2;   

DOI  :  10.1016/j.bbadis.2011.03.001

来源: Elsevier

【 摘 要 】

Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disease characterized by renal cyst formation and caused by mutations in the PKD1 and PKD2 genes, which encode polycystin-1(PC-1) and -2 (PC-2) proteins, respectively. PC-1 is a large plasma membrane receptor involved in the regulation of several biological functions and signaling pathways including the Wnt cascade, AP-1, PI3kinase/Akt, GSK3 beta, STAT6, Calcineurin/NFAT and the ERK and mTOR cascades. PC-2 is a calcium channel of the TRP family. The two proteins form a functional complex and prevent cyst formation, but the precise mechanism(s) involved remains unknown. This article is part of a Special Issue entitled: Polycystic Kidney Disease. Published by Elsevier B.V.

【 授权许可】

Free