Mitochondrial disorders are among the most frequent inborn errors of metabolism, their
primary cause being the dysfunction of the oxidative phosphorylation system (OXPHOS) …

Mitochondria are double‐membrane‐bound organelles that are present in all nucleated
eukaryotic cells and are responsible for the production of cellular energy in the form of ATP …

Complex I (NADH: ubiquinone oxidoreductase) is the largest multimeric enzyme complex of
the mitochondrial respiratory chain, which is responsible for electron transport and the …

Leigh syndrome is a rare, complex, and incurable early onset (typically infant or early
childhood) mitochondrial disorder with both phenotypic and genetic heterogeneity. The …

Mitochondria are ubiquitous organelles in eukaryotic cells whose primary function is to
generate energy supplies in the form of ATP through oxidative phosphorylation. As the entry …

Leigh syndrome (also termed subacute, necrotizing encephalopathy) is a devastating
neurodegenerative disorder, characterized by almost identical brain changes, eg, focal …

GLS1 orchestrates glutaminolysis and promotes cell proliferation when glutamine is
abundant by regenerating TCA cycle intermediates and supporting redox homeostasis. CB …

Mitochondria are the primary source of energy production and are implicated in a wide
range of biological processes in most eukaryotic cells. Skeletal muscle heavily relies on …

Mitochondrial DNA (mtDNA)-associated Leigh syndrome and NARP (neurogenic muscle
weakness, ataxia, and retinitis pigmentosa) are part of a continuum of progressive …

Assembly of the oxidative phosphorylation (OXPHOS) system in the mitochondrial inner
membrane is an intricate process in which many factors must interact. The OXPHOS system …