Translational research in ADPKD: lessons from animal models

The aging kidney revisited: A systematic review

(2014) Davide Bolignano et al.   AGEING RESEARCH REVIEWS

Autosomal dominant polycystic kidney disease

(2013) C. L. Chow et al.   CLINICAL MEDICINE

Multiple postnatal craniofacial anomalies are characterized by conditional loss of polycystic kidney disease 2 (Pkd2)

(2013) Roman H. Khonsari et al.   HUMAN MOLECULAR GENETICS

Hyperphosphorylation of polycystin-2 at a critical residue in disease reveals an essential role for polycystin-1-regulated dephosphorylation

(2013) Andrew J. Streets et al.   HUMAN MOLECULAR GENETICS

Progressive development of polycystic kidney disease in the mouse model expressing Pkd1 extracellular domain

(2013) Almira Kurbegovic et al.   HUMAN MOLECULAR GENETICS

Proximal Tubule Proliferation Is Insufficient to Induce Rapid Cyst Formation after Cilia Disruption

(2013) N. Sharma et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

A Pkd1-Fbn1 Genetic Interaction Implicates TGF-  Signaling in the Pathogenesis of Vascular Complications in Autosomal Dominant Polycystic Kidney Disease

(2013) D. Liu et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Type of PKD1 Mutation Influences Renal Outcome in ADPKD

(2013) E. Cornec-Le Gall et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Cyst expansion and regression in a mouse model of polycystic kidney disease

(2013) Hester Happé et al.   KIDNEY INTERNATIONAL

Macrophages promote polycystic kidney disease progression

(2013) Katherine I Swenson-Fields et al.   KIDNEY INTERNATIONAL

Effect of longacting somatostatin analogue on kidney and cyst growth in autosomal dominant polycystic kidney disease (ALADIN): a randomised, placebo-controlled, multicentre trial

(2013) Anna Caroli et al.   LANCET

ANKS6 is a central component of a nephronophthisis module linking NEK8 to INVS and NPHP3

(2013) Sylvia Hoff et al.   NATURE GENETICS

Loss of cilia suppresses cyst growth in genetic models of autosomal dominant polycystic kidney disease

(2013) Ming Ma et al.   NATURE GENETICS

Defective glucose metabolism in polycystic kidney disease identifies a new therapeutic strategy

(2013) Isaline Rowe et al.   NATURE MEDICINE

Role of interstitial inflammation in the pathogenesis of polycystic kidney disease

(2013) Michelle HT Ta et al.   NEPHROLOGY

miR-17 92 miRNA cluster promotes kidney cyst growth in polycystic kidney disease

(2013) V. Patel et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Chronic asymptomatic pyuria precedes overt urinary tract infection and deterioration of renal function in autosomal dominant polycystic kidney disease

(2013) Jin Ho Hwang et al.   BMC Nephrology

Renal replacement therapy in ADPKD patients: a 25-year survey based on the Catalan registry

(2013) Víctor Martínez et al.   BMC Nephrology

The role of the cilium in normal and abnormal cell cycles: emphasis on renal cystic pathologies

(2012) Junmin Pan et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Functional polycystin-1 dosage governs autosomal dominant polycystic kidney disease severity

(2012) Katharina Hopp et al.   JOURNAL OF CLINICAL INVESTIGATION

Tolvaptan in Patients with Autosomal Dominant Polycystic Kidney Disease

(2012) Vicente E. Torres et al.   NEW ENGLAND JOURNAL OF MEDICINE

Prenatal diagnosis and molecular genetic analysis of short rib-polydactyly syndrome type III (Verma-Naumoff) in a second-trimester fetus with a homozygous splice site mutation in intron 4 in the NEK1 gene

(2012) Chih-Ping Chen et al.   Taiwanese Journal of Obstetrics & Gynecology

Mechanoprotection by Polycystins against Apoptosis Is Mediated through the Opening of Stretch-Activated K2P Channels

(2012) Rémi Peyronnet et al.   Cell Reports

NEK1 Mutations Cause Short-Rib Polydactyly Syndrome Type Majewski

(2011) Christian Thiel et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Polycystic kidney disease: The complexity of planar cell polarity and signaling during tissue regeneration and cyst formation

(2011) Hester Happé et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Cilium, centrosome and cell cycle regulation in polycystic kidney disease

(2011) Kyung Lee et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Polycystins, focal adhesions and extracellular matrix interactions

(2011) Iain A. Drummond   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Two mutations in human BICC1 resulting in Wnt pathway hyperactivity associated with cystic renal dysplasia

(2011) Marine R.-C. Kraus et al.   HUMAN MUTATION

Neonatal onset autosomal dominant polycystic kidney disease (ADPKD) in a patient homozygous for aPKD2missense mutation due to uniparental disomy

(2011) M Losekoot et al.   JOURNAL OF MEDICAL GENETICS

Altered Hippo signalling in polycystic kidney disease

(2011) Hester Happé et al.   JOURNAL OF PATHOLOGY

Epitope-Tagged Pkhd1 Tracks the Processing, Secretion, and Localization of Fibrocystin

(2011) J. L. Bakeberg et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Macrophages Promote Cyst Growth in Polycystic Kidney Disease

(2011) A. Karihaloo et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Mutations in Multiple PKD Genes May Explain Early and Severe Polycystic Kidney Disease

(2011) C. Bergmann et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

A genetic interaction network of five genes for human polycystic kidney and liver diseases defines polycystin-1 as the central determinant of cyst formation

(2011) Sorin V Fedeles et al.   NATURE GENETICS

Therapeutic potential of vasopressin V2 receptor antagonist in a mouse model for autosomal dominant polycystic kidney disease: optimal timing and dosing of the drug

(2011) E. Meijer et al.   NEPHROLOGY DIALYSIS TRANSPLANTATION

Mechanism-Based Therapeutics for Autosomal Dominant Polycystic Kidney Disease: Recent Progress and Future Prospects

(2011) Ming-Yang Chang et al.   NEPHRON CLINICAL PRACTICE

US Renal Data System 2010 Annual Data Report

(2010) Allan J. Collins et al.   AMERICAN JOURNAL OF KIDNEY DISEASES

Evidence of Extraordinary Growth in the Progressive Enlargement of Renal Cysts

(2010) J. J. Grantham et al.   Clinical Journal of the American Society of Nephrology

The RNA-binding protein bicaudal C regulates polycystin 2 in the kidney by antagonizing miR-17 activity

(2010) U. Tran et al.   DEVELOPMENT

Cystogenesis in ARPKD results from increased apoptosis in collecting duct epithelial cells of Pkhd1 mutant kidneys

(2010) Bo Hu et al.   EXPERIMENTAL CELL RESEARCH

Pkd1 transgenic mice: adult model of polycystic kidney disease with extrarenal and renal phenotypes

(2010) Almira Kurbegovic et al.   HUMAN MOLECULAR GENETICS

Progressive renal distortion by multiple cysts in transgenic mice expressing artificial microRNAs against Pkd1

(2010) Ellian Wang et al.   JOURNAL OF PATHOLOGY

Randomized Clinical Trial of Long-Acting Somatostatin for Autosomal Dominant Polycystic Kidney and Liver Disease

(2010) M. C. Hogan et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Incompletely Penetrant PKD1 Alleles Mimic the Renal Manifestations of ARPKD

(2010) M. Vujic et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Aberrant Regulation of Planar Cell Polarity in Polycystic Kidney Disease

(2010) A. Luyten et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Pkd1-inactivation in vascular smooth muscle cells and adaptation to hypertension

(2010) Sabrine Hassane et al.   LABORATORY INVESTIGATION

Coupling between hydrodynamic forces and planar cell polarity orients mammalian motile cilia

(2010) Boris Guirao et al.   NATURE CELL BIOLOGY

Primary cilia regulate mTORC1 activity and cell size through Lkb1

(2010) Christopher Boehlke et al.   NATURE CELL BIOLOGY

Inhibition of glucosylceramide accumulation results in effective blockade of polycystic kidney disease in mouse models

(2010) Thomas A Natoli et al.   NATURE MEDICINE

Inhibition of mTOR with sirolimus does not attenuate progression of liver and kidney disease in PCK rats

(2010) C. Renken et al.   NEPHROLOGY DIALYSIS TRANSPLANTATION

Sirolimus and Kidney Growth in Autosomal Dominant Polycystic Kidney Disease

(2010) Andreas L. Serra et al.   NEW ENGLAND JOURNAL OF MEDICINE

Everolimus in Patients with Autosomal Dominant Polycystic Kidney Disease

(2010) Gerd Walz et al.   NEW ENGLAND JOURNAL OF MEDICINE

Pkd1 and Pkd2 Are Required for Normal Placental Development

(2010) Miguel A. Garcia-Gonzalez et al.   PLoS One

Pkd2 Dosage Influences Cellular Repair Responses following Ischemia-Reperfusion Injury

(2009) Sony Prasad et al.   AMERICAN JOURNAL OF PATHOLOGY

Polycystin-1 and -2 Dosage Regulates Pressure Sensing

(2009) Reza Sharif-Naeini et al.   CELL

Lanreotide Reduces the Volume of Polycystic Liver: A Randomized, Double-Blind, Placebo-Controlled Trial

(2009) Loes van Keimpema et al.   GASTROENTEROLOGY

ERK1/2-Dependent Vascular Endothelial Growth Factor Signaling Sustains Cyst Growth in Polycystin-2 Defective Mice

(2009) Carlo Spirli et al.   GASTROENTEROLOGY

Toxic tubular injury in kidneys from Pkd1-deletion mice accelerates cystogenesis accompanied by dysregulated planar cell polarity and canonical Wnt signaling pathways

(2009) H. Happe et al.   HUMAN MOLECULAR GENETICS

Renal injury is a third hit promoting rapid development of adult polycystic kidney disease

(2009) Ayumi Takakura et al.   HUMAN MOLECULAR GENETICS

Systems biology of autosomal dominant polycystic kidney disease (ADPKD): computational identification of gene expression pathways and integrated regulatory networks

(2009) Xuewen Song et al.   HUMAN MOLECULAR GENETICS

Pkd1 Haploinsufficiency Increases Renal Damage and Induces Microcyst Formation following Ischemia/Reperfusion

(2009) A. P. Bastos et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Loss of Oriented Cell Division Does not Initiate Cyst Formation

(2009) S. Nishio et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Conditional Mutation of Pkd2 Causes Cystogenesis and Upregulates  -Catenin

(2009) I. Kim et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Inactivation of Pkd1 in principal cells causes a more severe cystic kidney disease than in intercalated cells

(2009) Kalani L. Raphael et al.   KIDNEY INTERNATIONAL

Incompletely penetrant PKD1 alleles suggest a role for gene dosage in cyst initiation in polycystic kidney disease

(2009) Sandro Rossetti et al.   KIDNEY INTERNATIONAL

Autosomal dominant polycystic kidney disease: the last 3 years

(2009) Vicente E. Torres et al.   KIDNEY INTERNATIONAL

A mitotic transcriptional switch in polycystic kidney disease

(2009) Francisco Verdeguer et al.   NATURE MEDICINE

Cardiovascular abnormalities in autosomal-dominant polycystic kidney disease

(2009) Tevfik Ecder et al.   Nature Reviews Nephrology

A Novel Mouse Model Reveals that Polycystin-1 Deficiency in Ependyma and Choroid Plexus Results in Dysfunctional Cilia and Hydrocephalus

(2009) Claas Wodarczyk et al.   PLoS One

Loss of Nephrocystin-3 Function Can Cause Embryonic Lethality, Meckel-Gruber-like Syndrome, Situs Inversus, and Renal-Hepatic-Pancreatic Dysplasia

(2008) Carsten Bergmann et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Biliary and Pancreatic Dysgenesis in Mice Harboring a Mutation in Pkhd1

(2008) Anna-Rachel Gallagher et al.   AMERICAN JOURNAL OF PATHOLOGY

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

(2008) Vishal Patel et al.   HUMAN MOLECULAR GENETICS

Cyst formation and activation of the extracellular regulated kinase pathway after kidney specific inactivation of Pkd1

(2008) Sekiya Shibazaki et al.   HUMAN MOLECULAR GENETICS

Polycystin-1 C-terminal tail associates with β-catenin and inhibits canonical Wnt signaling

(2008) Mark Lal et al.   HUMAN MOLECULAR GENETICS

Cyst Formation in Kidney via B-Raf Signaling in thePKD2Transgenic Mice

(2008) Eun Young Park et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Polycystin-2 Expression Is Regulated by a PC2-binding Domain in the Intracellular Portion of Fibrocystin

(2008) Ingyu Kim et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Deletion of IFT20 in the mouse kidney causes misorientation of the mitotic spindle and cystic kidney disease

(2008) Julie A. Jonassen et al.   JOURNAL OF CELL BIOLOGY

Fibrocystin/Polyductin Modulates Renal Tubular Formation by Regulating Polycystin-2 Expression and Function

(2008) I. Kim et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Pkd1 Inactivation Induced in Adulthood Produces Focal Cystic Disease

(2008) A. Takakura et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

NEK8 Mutations Affect Ciliary and Centrosomal Localization and May Cause Nephronophthisis

(2008) E. A. Otto et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

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

(2008) P.G. Starremans et al.   KIDNEY INTERNATIONAL

Loss of Fat4 disrupts PCP signaling and oriented cell division and leads to cystic kidney disease

(2008) Sakura Saburi et al.   NATURE GENETICS

Kidney cysts, pancreatic cysts, and biliary disease in a mouse model of autosomal recessive polycystic kidney disease

(2008) Scott S. Williams et al.   PEDIATRIC NEPHROLOGY

Vasopressin Directly Regulates Cyst Growth in Polycystic Kidney Disease

(2007) X. Wang et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Innate immunity as a driving force in renal disease

(2007) E. de Heer et al.   KIDNEY INTERNATIONAL

Overexpression of innate immune response genes in a model of recessive polycystic kidney disease

(2007) M. Mrug et al.   KIDNEY INTERNATIONAL

Autosomal Dominant Polycystic Kidney Disease: Recent Advances in Pathogenesis and Treatment

(2007) Ming-Yang Chang et al.   NEPHRON PHYSIOLOGY