Podocytopathies are kidney diseases in which direct or indirect podocyte injury drives
proteinuria or nephrotic syndrome. In children and young adults, genetic variants in> 50 …

The glomerular basement membrane (GBM) is a key component of the glomerular capillary
wall and is essential for kidney filtration. The major components of the GBM include …

Early kidney failure in the hereditary type IV collagen disease, Alport syndrome, can be
delayed by renin-angiotensin inhibitors. However, whether all patients and all different …

Currently, there are no treatments for Alport syndrome, which is the second most commonly
inherited kidney disease. Here we report the development of an exon-skipping therapy …

Alport syndrome is a hereditary disorder characterized by kidney disease, ocular
abnormalities, and sensorineural hearing loss. Work in understanding the cause of Alport …

Anti-glomerular basement membrane (anti-GBM) disease is a rare life-threatening small-
vessel vasculitis that typically affects the capillaries of kidneys and lungs, with most of …

The basement membrane (BM) is a specialized layer of extracellular matrix components that
plays a central role in maintaining lung and kidney functions. Although the composition of …

A study of 269 children enrolled into a National Registry for children with persistent
glomerular hematuria identified 131 individuals with genetically confirmed X-linked Alport …

Podocytes form the backbone of the glomerular filtration barrier and are exposed to various
mechanical forces throughout the lifetime of an individual. The highly dynamic …

Chronic kidney disease (CKD) triggers the risk of developing uremic cardiomyopathy as
characterized by cardiac hypertrophy, fibrosis and functional impairment. Traditionally …