Modeling Renal Disease “On the Fly”

Bicaudal C mutation causes myc and TOR pathway up-regulation and polycystic kidney disease-like phenotypes in Drosophila

(2017) Chiara Gamberi et al.   PLoS Genetics

Using the Drosophila Nephrocyte to Model Podocyte Function and Disease

(2017) Martin Helmstädter et al.   Frontiers in Pediatrics

Will you still need me (Ca2+ , TnT, and DHPR), will you still cleave me (calpain), when I'm 64?

(2016) José Renato Pinto et al.   AGING CELL

Modeling Monogenic Human Nephrotic Syndrome in theDrosophilaGarland Cell Nephrocyte

(2016) Tobias Hermle et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Ecdysone regulates morphogenesis and function of malpighian tubules in Drosophila melanogaster through EcR-B2 isoform

(2015) Naveen Kumar Gautam et al.   DEVELOPMENTAL BIOLOGY

Anks3 interacts with nephronophthisis proteins and is required for normal renal development

(2015) Toma A. Yakulov et al.   KIDNEY INTERNATIONAL

A Drosophila Model Identifies a Critical Role for Zinc in Mineralization for Kidney Stone Disease

(2015) Thomas Chi et al.   PLoS One

Klf15 Is Critical for the Development and Differentiation of Drosophila Nephrocytes

(2015) Jessica R. Ivy et al.   PLoS One

Translational research in ADPKD: lessons from animal models

(2014) Hester Happé et al.   Nature Reviews Nephrology

Src64B phosphorylates Dumbfounded and regulates slit diaphragm dynamics: Drosophila as a model to study nephropathies

(2013) A. S. Tutor et al.   DEVELOPMENT

HNF1B controls proximal-intermediate nephron segment identity in vertebrates by regulating Notch signalling components and Irx1/2

(2013) C. Heliot et al.   DEVELOPMENT

Ecdysone triggered PGRP-LC expression controls Drosophila innate immunity

(2013) Florentina Rus et al.   EMBO JOURNAL

Epithelial Immune Response inDrosophilaMalpighian Tubules: Interplay Between Diap2 and Ion Channels

(2013) Puja Verma et al.   JOURNAL OF CELLULAR PHYSIOLOGY

The Drosophila Nephrocyte: Back on Stage

(2013) J. Na et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Drosophila melanogaster as an Emerging Translational Model of Human Nephrolithiasis

(2013) Joe Miller et al.   JOURNAL OF UROLOGY

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

(2013) Hester Happé et al.   KIDNEY INTERNATIONAL

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

(2013) Isaline Rowe et al.   NATURE MEDICINE

The Bic-C Family of Developmental Translational Regulators

(2012) Chiara Gamberi et al.   COMPARATIVE AND FUNCTIONAL GENOMICS

Tissue- and Ligand-Specific Sensing of Gram-Negative Infection in Drosophila by PGRP-LC Isoforms and PGRP-LE

(2012) C. Neyen et al.   JOURNAL OF IMMUNOLOGY

Immune and stress response ‘cross-talk’ in the Drosophila Malpighian tubule

(2012) Shireen-Anne Davies et al.   JOURNAL OF INSECT PHYSIOLOGY

SIX2 and CITED1, markers of nephronic progenitor self-renewal, remain active in primitive elements of Wilms' tumor

(2012) Andrew J. Murphy et al.   JOURNAL OF PEDIATRIC SURGERY

Dose-Dependent Effects of Sirolimus on mTOR Signaling and Polycystic Kidney Disease

(2012) Z. Novalic et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Cubilin and Amnionless Mediate Protein Reabsorption in Drosophila Nephrocytes

(2012) F. Zhang et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Functional Correlates of Positional and Gender-Specific Renal Asymmetry in Drosophila

(2012) Venkateswara R. Chintapalli et al.   PLoS One

Correction: Immune Response and Anti-Microbial Peptides Expression in Malpighian Tubules of Drosophila melanogaster Is under Developmental Regulation

(2012) Puja Verma et al.   PLoS One

Bicc1 links the regulation of cAMP signaling in polycystic kidneys to microRNA-induced gene silencing

(2012) Nathalie Piazzon et al.   Journal of Molecular Cell Biology

Lgr5+ve Stem/Progenitor Cells Contribute to Nephron Formation during Kidney Development

(2012) Nick Barker et al.   Cell Reports

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

The Drosophila nephrocyte

(2011) Ross L Cagan   CURRENT OPINION IN NEPHROLOGY AND HYPERTENSION

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

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

Altered Hippo signalling in polycystic kidney disease

(2011) Hester Happé et al.   JOURNAL OF PATHOLOGY

Ethylene glycol induces calcium oxalate crystal deposition in Malpighian tubules: a Drosophila model for nephrolithiasis/urolithiasis

(2011) Yung-Hsiang Chen et al.   KIDNEY INTERNATIONAL

Ciliopathies

(2011) Friedhelm Hildebrandt et al.   NEW ENGLAND JOURNAL OF MEDICINE

Semaphorins in kidney development and disease: modulators of ureteric bud branching, vascular morphogenesis, and podocyte-endothelial crosstalk

(2011) Kimberly Reidy et al.   PEDIATRIC NEPHROLOGY

Cellular Maintenance and Repair of the Kidney

(2010) Jian-Kan Guo et al.   Annual Review of Physiology

Renal Function and Healthcare Costs in Patients with Polycystic Kidney Disease

(2010) K. L. Lentine 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

Loss of Bicc1 impairs tubulomorphogenesis of cultured IMCD cells by disrupting E-cadherin-based cell-cell adhesion

(2010) Yulong Fu et al.   EUROPEAN JOURNAL OF CELL BIOLOGY

Rapamycin Ameliorates PKD Resulting from Conditional Inactivation of Pkd1

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

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

The many faces of RET dysfunction in kidney

(2010) Sanjay Jain   Organogenesis

Fate Tracing Reveals the Pericyte and Not Epithelial Origin of Myofibroblasts in Kidney Fibrosis

(2009) Benjamin D. Humphreys et al.   AMERICAN JOURNAL OF PATHOLOGY

The polycystic kidney disease-related proteins Bicc1 and SamCystin interact

(2009) Emily E. Stagner et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Sns and Kirre, the Drosophila orthologs of Nephrin and Neph1, direct adhesion, fusion and formation of a slit diaphragm-like structure in insect nephrocytes

(2009) S. Zhuang et al.   DEVELOPMENT

Bicaudal C, a novel regulator of Dvl signaling abutting RNA-processing bodies, controls cilia orientation and leftward flow

(2009) C. Maisonneuve et al.   DEVELOPMENT

Bicaudal C and trailer hitch have similar roles in gurken mRNA localization and cytoskeletal organization

(2009) Mark J. Snee et al.   DEVELOPMENTAL BIOLOGY

Bicaudal-C associates with a Trailer Hitch/Me31B complex and is required for efficient Gurken secretion

(2009) Jan-Michael Kugler et al.   DEVELOPMENTAL BIOLOGY

Analysis of early nephron patterning reveals a role for distal RV proliferation in fusion to the ureteric tip via a cap mesenchyme-derived connecting segment

(2009) Kylie Georgas et al.   DEVELOPMENTAL BIOLOGY

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

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

Calcimimetic Inhibits Late-Stage Cyst Growth in ADPKD

(2009) V. H. Gattone et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Flying podocytes

(2009) Matias Simons et al.   KIDNEY INTERNATIONAL

Late progression of renal pathology and cyst enlargement is reduced by rapamycin in a mouse model of nephronophthisis

(2009) Vincent H. Gattone 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

Polycystin-1 Regulates Extracellular Signal-Regulated Kinase-Dependent Phosphorylation of Tuberin To Control Cell Size through mTOR and Its Downstream Effectors S6K and 4EBP1

(2009) G. Distefano et al.   MOLECULAR AND CELLULAR BIOLOGY

Polycystic Kidney Disease

(2008) Peter C. Harris et al.   Annual Review of Medicine

Six2 Defines and Regulates a Multipotent Self-Renewing Nephron Progenitor Population throughout Mammalian Kidney Development

(2008) Akio Kobayashi et al.   Cell Stem Cell

Osr1 expression demarcates a multi-potent population of intermediate mesoderm that undergoes progressive restriction to an Osr1-dependent nephron progenitor compartment within the mammalian kidney

(2008) Joshua W. Mugford et al.   DEVELOPMENTAL BIOLOGY

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

(2008) Mark Lal et al.   HUMAN MOLECULAR GENETICS

The insect nephrocyte is a podocyte-like cell with a filtration slit diaphragm

(2008) Helen Weavers et al.   NATURE