Abstract Coenzyme Q10 (CoQ10) is an essential electron carrier in the mitochondrial
respiratory chain and an important antioxidant. Deficiency of CoQ10 is a clinically and …

Renal proximal tubules are highly sensitive to ischemic and toxic insults and are affected in
diverse genetic disorders, of which nephropathic cystinosis is the most common. The …

Abstract Coenzyme Q 10 (CoQ 10) is essential for electron transport in the mitochondrial
respiratory chain and antioxidant defense. The relative importance of respiratory chain …

Mutations in NPHS1, which encodes nephrin, are the main causes of congenital nephrotic
syndrome (CNS) in Finnish patients, whereas mutations in NPHS2, which encodes podocin …

Mitochondrial dysfunction contributes to podocyte injury, but normal podocyte bioenergetics
have not been characterized. We measured oxygen consumption rates (OCR) and …

Background Coenzyme Q10 (CoQ10) and its analogs are used therapeutically by virtue of
their functions as electron carriers, antioxidant compounds, or both. However, published …

The glomerulus consists of capillary tufts, a mesangial cell component and the Bowman
capsule. The glomerular filtration barrier is composed of glomerular endothelial cells, a …

The recent discovery of genes involved in familial forms of nephrotic syndrome represents a
break-through in nephrology. To date, 15 genes have been characterized and several new …

The mechanism responsible for proteinuria in non-genetic idiopathic nephrotic syndrome
(iNS) is unknown. Animal models suggest an effect of free radicals on podocytes, and …

A Mendelian inheritance underlies a nonnegligible proportion of hereditary kidney diseases,
suggesting that the encoded proteins are essential for maintenance of the renal function …