Acute kidney injury and aberrant planar cell polarity induce cyst formation in mice lacking renal cilia

V Patel, L Li, P Cobo-Stark, X Shao… - Human molecular …, 2008 - academic.oup.com
Polycystic kidney disease (PKD) is an inherited disorder that is characterized by the
accumulation of cysts in the renal parenchyma and progressive decline in renal function …

Advances in the pathogenesis and treatment of polycystic kidney disease

V Patel, R Chowdhury, P Igarashi - Current opinion in nephrology …, 2009 - journals.lww.com
Abnormalities in the primary cilium perturb signaling pathways that regulate renal epithelial
cell growth and differentiation and lead to the formation of kidney cysts. Acute kidney injury …

Development of polycystic kidney disease in juvenile cystic kidney mice: insights into pathogenesis, ciliary abnormalities, and common features with human disease

LA Smith, NO Bukanov, H Husson… - Journal of the …, 2006 - journals.lww.com
Significant progress in understanding the molecular mechanisms of polycystic kidney
disease (PKD) has been made in recent years. Translating this understanding into effective …

Loss of primary cilia upregulates renal hypertrophic signaling and promotes cystogenesis

PD Bell, W Fitzgibbon, K Sas, AE Stenbit… - Journal of the …, 2011 - journals.lww.com
Primary cilia dysfunction alters renal tubular cell proliferation and differentiation and
associates with accelerated cyst formation in polycystic kidney disease. However, the …

Cilia and polycystic kidney disease

M Ma - Seminars in Cell & Developmental Biology, 2021 - Elsevier
Polycystic kidney disease (PKD), comprising autosomal dominant polycystic kidney disease
(ADPKD) and autosomal recessive polycystic kidney d isease (ARPKD), is characterized by …

Cystin, a novel cilia-associated protein, is disrupted in the cpk mouse model of polycystic kidney disease

X Hou, M Mrug, BK Yoder, EJ Lefkowitz… - The Journal of …, 2002 - Am Soc Clin Investig
The congenital polycystic kidney (cpk) mutation is the most extensively characterized mouse
model of polycystic kidney disease (PKD). The renal cystic disease is fully expressed in …

[HTML][HTML] Delayed cystogenesis and increased ciliogenesis associated with the re-expression of polaris in Tg737 mutant mice

NE Brown, NS Murcia - Kidney international, 2003 - Elsevier
Delayed cystogenesis and increased ciliogenesis associated with the re-expression of
polaris inTg737 mutant mice. Background Renal cysts and shortened cilia on renal tubular …

Role of primary cilia in the pathogenesis of polycystic kidney disease

BK Yoder - Journal of the American Society of Nephrology, 2007 - journals.lww.com
Cysts in the kidney are among the most common inherited human pathologies, and recent
research has uncovered that a defect in cilia-mediated signaling activity is a key factor that …

[HTML][HTML] Zebrafish mutations affecting cilia motility share similar cystic phenotypes and suggest a mechanism of cyst formation that differs from pkd2 morphants

J Sullivan-Brown, J Schottenfeld, N Okabe… - Developmental …, 2008 - Elsevier
Zebrafish are an attractive model for studying the earliest cellular defects occurring during
renal cyst formation because its kidney (the pronephros) is simple and genes that cause …

Polycystin-2 localizes to kidney cilia and the ciliary level is elevated in orpk mice with polycystic kidney disease

GJ Pazour, JT San Agustin, JA Follit, JL Rosenbaum… - Current Biology, 2002 - cell.com
Defects in the PKD1 and PKD2 genes cause autosomal dominant polycystic kidney disease
(PKD) in~ 1 in 1000 adults worldwide. These genes encode polycystin-1 and polycystin-2 …