Dihydrolipoamide dehydrogenase (DLD) deficiency is a recessive mitochondrial disorder
caused by depletion of DLD from α-ketoacid dehydrogenase complexes. Caenorhabditis …

Pathogenic variants in the human F-box and leucine-rich repeat protein 4 (FBXL4) gene
result in an autosomal recessive, multisystemic, mitochondrial disorder involving variable …

Mitochondrial dysfunction, with an estimated incidence of 1 in 5,000 births, is associated
with a wide variety of multisystem degenerative diseases. Among the most prevalent forms …

Mitochondrial respiratory chain disorders are empirically managed with variable antioxidant,
cofactor and vitamin 'cocktails'. However, clinical trial validated and approved compounds …

In Caenorhabditis elegans, slow fat consumption has been suggested to contribute to the
extension of the survival rate during nutritionally adverse conditions. Here, we investigated …

Mitochondria are known as the powerhouse of the cell. Dysfunction of mitochondria
homeostasis induces the mitochondrial unfolded protein response (UPR mt), altering …

Mutations in the clk-1 gene impair mitochondrial ubiquinone biosynthesis and extend the
lifespan in Caenorhabditis elegans. We demonstrate here that this life extension is linked to …

The Dld gene product, known as dihydrolipoamide dehydrogenase or the E3 component,
catalyzes the oxidation of dihydrolipoyl moieties of four mitochondrial multienzyme …

The NADH: ubiquinone oxidoreductase or complex I of the mitochondrial respiratory chain is
an intricate enzyme with a vital role in energy metabolism. Mutations affecting complex I can …

In Caenorhabditis elegans, mitochondrial dysfunction caused by mutation or toxins activates
programs of detoxification and immune response. A genetic screen for mutations that …