Contrary to their well-known functions in nutrient breakdown, mitochondria are also
important biosynthetic hubs and express an evolutionarily conserved mitochondrial fatty acid …

Abstract In 2016, the Movement Disorder Society Task Force for the Nomenclature of
Genetic Movement Disorders presented a new system for naming genetically determined …

In most eukaryotic cells, fatty acid synthesis (FAS) occurs in the cytoplasm and in
mitochondria. However, the relative contribution of mitochondrial FAS (mtFAS) to the cellular …

Cells harbor two systems for fatty acid synthesis, one in the cytoplasm (catalyzed by fatty
acid synthase, FASN) and one in the mitochondria (mtFAS). In contrast to FASN, mtFAS is …

Background Leber's hereditary optic neuropathy (LHON) is a mitochondrial disorder
characterised by complex I defect leading to sudden degeneration of retinal ganglion cells …

Optic atrophy (OA) with autosomal inheritance is a form of optic neuropathy characterized by
the progressive and irreversible loss of vision. In some cases, this is accompanied by …

Fatty acids in mitochondria, in sensu stricto, arise either as β-oxidation substrates imported
via the carnitine shuttle or through de novo synthesis by the mitochondrial fatty acid …

Childhood-onset dystonia with optic atrophy and basal ganglia abnormalities is an
extremely rare autosomal recessive mitochondrial disease caused by biallelic mutations in …

Introduction Mitochondrial Enoyl CoA Reductase Protein-Associated Neurodegeneration
(MEPAN) syndrome is a rare inherited metabolic condition caused by MECR gene …

In most eukaryotic cells fatty acid synthesis occurs in the cytoplasm as well as in
mitochondria. However, the relative contribution of mitochondrial fatty acid synthesis …