Murine models of polycystic kidney disease: molecular and therapeutic insights

LM Guay-Woodford - American Journal of Physiology-Renal …, 2003 - journals.physiology.org
Numerous murine (mouse and rat) models of polycystic kidney disease (PKD) have been
described in which the mutant phenotype results from a spontaneous mutation or …

New insights into polycystic kidney disease and its treatment

VE Torres - Current opinion in nephrology and hypertension, 1998 - journals.lww.com
Major advances in the understanding of the genetics and pathogenesis of autosomal
dominant polycystic kidney disease have occurred within the past year. The proteins …

Translational research in ADPKD: lessons from animal models

H Happe, DJM Peters - Nature Reviews Nephrology, 2014 - nature.com
Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in PKD1 or
PKD2, which encode polycystin-1 and polycystin-2, respectively. Rodent models are …

[HTML][HTML] New insights into the molecular pathophysiology of polycystic kidney disease

NS Murcia, WE Sweeney Jr, ED Avner - Kidney international, 1999 - Elsevier
New insights into the molecular pathophysiology of polycystic kidney disease. Polycystic
kidney diseases are characterized by the progressive expansion of multiple cystic lesions …

[HTML][HTML] A mouse model for polycystic kidney disease through a somatic in-frame deletion in the 5′ end of Pkd1

PG Starremans, X Li, PE Finnerty, L Guo, A Takakura… - Kidney international, 2008 - Elsevier
Autosomal dominant polycystic kidney disease, a leading cause of end-stage renal disease
in adults, is characterized by progressive focal cyst formation in the kidney. Embryonic …

Modification of disease progression in rats with inherited polycystic kidney disease

BD Cowley Jr, JJ Grantham, MJ Muessel… - American journal of …, 1996 - Elsevier
The most common inherited form of human polycystic kidney disease (PKD), autosomal
dominant PKD (ADPKD), is a leading cause of chronic renal failure, but has a variable …

Autosomal dominant polycystic kidney disease: clues to pathogenesis

PC Harris - Human molecular genetics, 1999 - academic.oup.com
Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutation of one of two
genes: PKD1 (16p13. 3) or PKD2 (4q13–23). PKD1 accounts for∼ 85% of pedigrees and is …

Animal models for human polycystic kidney disease

S Nagao, M Kugita, D Yoshihara… - Experimental …, 2012 - jstage.jst.go.jp
Polycystic kidney disease (PKD) is a hereditary disorder with abnormal cellular proliferation,
fluid accumulation in numerous cysts, remodeling of extracellular matrix, inflammation, and …

The genetic background significantly impacts the severity of kidney cystic disease in the Pkd1RC/RC mouse model of autosomal dominant polycystic kidney disease

J Arroyo, D Escobar-Zarate, HH Wells, MM Constans… - Kidney international, 2021 - Elsevier
Autosomal dominant polycystic kidney disease (ADPKD), primarily due to PKD1 or PKD2
mutations, causes progressive kidney cyst development and kidney failure. There is …

Molecular pathogenesis of autosomal dominant polycystic kidney disease

BK Yoder, S Mulroy, H Eustace, C Boucher… - Expert reviews in …, 2006 - cambridge.org
Autosomal dominant polycystic kidney disease (ADPKD) is one of the commonest inherited
human disorders yet remains relatively unknown to the wider medical, scientific and public …