FAD-dependent oxidoreductase domain-containing protein 1 (FOXRED1)

FAD-dependent oxidoreductase domain-containing protein 1 (FOXRED1) is a mitochondrial enzyme and molecular chaperone involved in the assembly and stabilization of respiratory chain complex I (NADH:ubiquinone oxidoreductase), the first and largest enzyme of the electron transport chain[3][5][6][8]. FOXRED1 contains a FAD-binding oxidoreductase domain, is encoded by the nuclear genome, and is evolutionarily related to the d-amino acid oxidase family[5]. Its primary function is to promote the assembly and integrity of complex I during mid-to-late stages, a critical step in mitochondrial bioenergetics, redox balance, and ATP production[2][3][5][7]. Loss-of-function mutations in FOXRED1 cause complex I deficiency, leading to severe mitochondrial diseases, including Leigh syndrome, encephalopathy with lactic acidosis, cardiomyopathy, and other multisystemic phenotypes with variable severity and poor prognosis[4][7][8]. FOXRED1 dysfunction may also alter cellular oxidative stress responses and influence disease outcomes in certain cancers[5]. There are currently no known direct drugs targeting FOXRED1, but its essential role in mitochondrial function makes it a potential target for therapeutic intervention and a biomarker for complex I-related disorders.

Other names

FOXRED1H17FP634MC1DN19FAD dependent oxidoreductase domain containing protein 1FAD-dependent oxidoreductase domain-containing protein 1FP-634

Restoration of complex I assembly or stabilization (inferred for potential therapeutic strategies targeting FOXRED1 dysfunction)