Alport syndrome: its effects on the glomerular filtration barrier and implications for future treatment

α1β1 Integrin/Rac1-Dependent Mesangial Invasion of Glomerular Capillaries in Alport Syndrome

(2013) Marisa Zallocchi et al.   AMERICAN JOURNAL OF PATHOLOGY

Evidence for Activation of the Unfolded Protein Response in Collagen IV Nephropathies

(2013) M. Pieri et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Expert Guidelines for the Management of Alport Syndrome and Thin Basement Membrane Nephropathy

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

The glomerular basement membrane as a barrier to albumin

(2013) Jung Hee Suh et al.   Nature Reviews Nephrology

Alport syndrome: the effects of spironolactone on proteinuria and urinary TGF-β1

(2013) Marisa Giani et al.   PEDIATRIC NEPHROLOGY

Phase 2a Study of Ataluren-Mediated Dystrophin Production in Patients with Nonsense Mutation Duchenne Muscular Dystrophy

(2013) Richard S. Finkel et al.   PLoS One

Inhibition of Integrin  2 1 Ameliorates Glomerular Injury

(2012) C. M. Borza et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Alport syndrome—insights from basic and clinical research

(2012) Jenny Kruegel et al.   Nature Reviews Nephrology

An update on the pathomechanisms and future therapies of Alport syndrome

(2012) Damien Noone et al.   PEDIATRIC NEPHROLOGY

Clinical utility gene card for: Alport syndrome

(2011) Jens Michael Hertz et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

An orally active chemokine receptor CCR2 antagonist prevents glomerulosclerosis and renal failure in type 2 diabetes

(2011) Sufyan G. Sayyed et al.   KIDNEY INTERNATIONAL

Early angiotensin-converting enzyme inhibition in Alport syndrome delays renal failure and improves life expectancy

(2011) Oliver Gross et al.   KIDNEY INTERNATIONAL

Biophysical properties of normal and diseased renal glomeruli

(2010) Hans M. Wyss et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Mapping structural landmarks, ligand binding sites, and missense mutations to the collagen IV heterotrimers predicts major functional domains, novel interactions, and variation in phenotypes in inherited diseases affecting basement membranes

(2010) J. Des Parkin et al.   HUMAN MUTATION

Loss of the BMP antagonist USAG-1 ameliorates disease in a mouse model of the progressive hereditary kidney disease Alport syndrome

(2010) Mari Tanaka et al.   JOURNAL OF CLINICAL INVESTIGATION

Electrical Forces Determine Glomerular Permeability

(2010) R. Hausmann et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Bacterial CpG-DNA accelerates Alport glomerulosclerosis by inducing an M1 macrophage phenotype and tumor necrosis factor-α-mediated podocyte loss

(2010) Mi Ryu et al.   KIDNEY INTERNATIONAL

Loss of collagen-receptor DDR1 delays renal fibrosis in hereditary type IV collagen disease

(2010) Oliver Gross et al.   MATRIX BIOLOGY

Tubular toxicity of proteinuria

(2010) Richard J. Baines et al.   Nature Reviews Nephrology

Update on the glomerular filtration barrier

(2009) George Jarad et al.   CURRENT OPINION IN NEPHROLOGY AND HYPERTENSION

Stem Cell Therapies Benefit Alport Syndrome

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

Cellular Origins of Type IV Collagen Networks in Developing Glomeruli

(2009) D. R. Abrahamson et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

C3a Mediates Epithelial-to-Mesenchymal Transition in Proteinuric Nephropathy

(2009) Z. Tang et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Biomechanical strain causes maladaptive gene regulation, contributing to Alport glomerular disease

(2009) Daniel T. Meehan et al.   KIDNEY INTERNATIONAL

Genetic diseases of connective tissues: cellular and extracellular effects of ECM mutations

(2009) John F. Bateman et al.   NATURE REVIEWS GENETICS

A Sulfilimine Bond Identified in Collagen IV

(2009) R. Vanacore et al.   SCIENCE

The COOH terminus of megalin regulates gene expression in opossum kidney proximal tubule cells

(2008) Yuanli Li et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Revisiting the glomerular charge barrier in the molecular era

(2008) Scott J Harvey et al.   CURRENT OPINION IN NEPHROLOGY AND HYPERTENSION

Irradiation Prolongs Survival of Alport Mice

(2008) K. Katayama et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Resolved: Normal Glomeruli Filter Nephrotic Levels of Albumin

(2008) W. D. Comper et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Fenestrated Glomerular Capillaries Are Unique

(2008) W. Kriz   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

The R229Q mutation in NPHS2 may predispose to proteinuria in thin-basement-membrane nephropathy

(2008) Stephen Tonna et al.   PEDIATRIC NEPHROLOGY

Inhibition of NF-κB-dependent Bcl-xL expression by clusterin promotes albumin-induced tubular cell apoptosis

(2007) O. Takase et al.   KIDNEY INTERNATIONAL