Fibrocystin/polyductin modulates renal tubular formation by regulating polycystin-2 expression and function

I Kim, Y Fu, K Hui, G Moeckel, W Mai, C Li… - Journal of the …, 2008 - journals.lww.com
Autosomal recessive polycystic kidney disease is caused by mutations in PKHD1, which
encodes the membrane-associated receptor-like protein fibrocystin/polyductin (FPC). FPC …

Identification and characterization of polycystin-2, thePKD2 gene product

Y Cai, Y Maeda, A Cedzich, VE Torres, G Wu… - Journal of Biological …, 1999 - ASBMB
PKD2, the second gene for the autosomal dominant polycystic kidney disease (ADPKD),
encodes a protein, polycystin-2, with predicted structural similarity to cation channel …

The pathogenesis of autosomal dominant polycystic kidney disease

M Sutters - Nephron Experimental Nephrology, 2006 - karger.com
In individuals with autosomal dominant polycystic kidney disease (ADPKD), renal function
deteriorates as the kidneys become replaced by multitudes of fluid-filled cysts. Although the …

Polycystin expression in the kidney and other tissues: complexity, consensus and controversy

ACM Ong - Nephron Experimental Nephrology, 2000 - karger.com
PKD1, the major gene mutated in autosomal dominant polycystic kidney disease, was
identified in 1994, and fully sequenced in 1995. The protein which it encodes, polycystin-1 …

Identification of phosphorylation sites in the PKD1-encoded protein C-terminal domain

HP Li, L Geng, CR Burrow, PD Wilson - Biochemical and biophysical …, 1999 - Elsevier
The PKD1-encoded protein,“polycystin-1”, has a large N-terminal extracellular portion,
multiple transmembrane domains, and a short intracellular C-terminal tail with four tyrosine …

ADPKD: molecular characterization and quest for treatment

S Horie - Clinical and experimental nephrology, 2005 - Springer
Autosomal-dominant polycystic kidney disease (ADPKD) is a common hereditary disease
that features multiple cystogenesis in various organs and vascular defects. The genes …

[HTML][HTML] A polycystin-centric view of cyst formation and disease: the polycystins revisited

ACM Ong, PC Harris - Kidney international, 2015 - Elsevier
It is 20 years since the identification of PKD1, the major gene mutated in autosomal
dominant polycystic kidney disease (ADPKD), followed closely by the cloning of PKD2 …

Down-regulation of Pkd2 by siRNAs suppresses cell–cell adhesion in the mouse melanoma cells

GH Bian, G Cao, XY Lv, QW Li, H Sun, Y Xiao… - Molecular biology …, 2010 - Springer
The Pkd2 gene encodes an integral protein (~ 130 kDa), named polycystin-2 (PC-2). PC-2 is
mainly involved in autosomal dominant polycystic kidney disease. Recently, polycystin …

Comparative analysis of the polycystic kidney disease 1 (PKD1) gene reveals an integral membrane glycoprotein with multiple evolutionary conserved domains

R Sandford, B Sgotto, S Aparicio… - Human molecular …, 1997 - academic.oup.com
PKD1 is the major locus of the common genetic disorder autosomal dominant polycystic
kidney disease (ADPKD). Analysis of the predicted protein sequence of the human PKD1 …

Polycystic kidney disease and renal injury repair: common pathways, fluid flow, and the function of polycystin-1

T Weimbs - American Journal of Physiology-Renal …, 2007 - journals.physiology.org
The root cause for most cases of autosomal-dominant polycystic kidney disease (ADPKD) is
mutations in the polycystin-1 (PC1) gene. While PC1 has been implicated in a perplexing …