Mitochondria are increasingly recognized as key players in genetic and acquired renal
diseases. Most mitochondrial cytopathies that cause renal symptoms are characterized by …

The primary causes of chronic kidney disease (CKD) in children differ from those of CKD in
adults. In the USA the most common diagnostic groups of renal disease that manifest before …

Ubiquinone (UQ; also known as coenzyme Q; CoQ) is a mobile component of the
mitochondrial electron transport chain, where it acts as a pro-oxidant in its ubisemiquinone …

Podocytes are highly specialized cells of the kidney glomerulus that wrap around capillaries
and that neighbor cells of the Bowman's capsule. When it comes to glomerular filtration …

Steroid-resistant nephrotic syndrome (SRNS) represents the second most frequent cause of
chronic kidney disease in the first three decades of life. It manifests histologically as focal …

Coenzyme Q (CoQ) is a component of the electron transport chain that participates in
aerobic cellular respiration to produce ATP. In addition, CoQ acts as an electron acceptor in …

Steroid-resistant nephrotic syndrome (SRNS) causes 15% of chronic kidney disease (CKD).
Here we show that recessive mutations in FAT1 cause a distinct renal disease entity in four …

The UbiA superfamily of intramembrane prenyltransferases catalyzes a key biosynthetic step
in the production of ubiquinones, menaquinones, plastoquinones, hemes, chlorophylls …

COQ2 (p-hydroxybenzoate polyprenyl transferase) encodes the enzyme required for the
second step of the final reaction sequence of Coenzyme Q10 (CoQ) biosynthesis. Its …

Nephrotic syndrome (NS) in children includes a diverse group of diseases that range from
genetic diseases without any immunological defects to causes that are primarily due to …