| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 8399218 | Mitochondrion | 2015 | 8 Pages |
Abstract
FOXRED1 mutations result in complex I (NADH:ubiquinone oxidoreductase) deficiencies and Leigh syndrome (subacute necrotizing encephalomyelopathy). FOXRED1 is a mitochondrial flavoprotein related to N-methyl amino acid dehydrogenases. How is FOXRED1 required for the biogenesis of complex I? I present a hypothesis that suggests FOXRED1 catalytic activity as a sarcosine oxidase protects the developing fetus from oxidative stress during pregnancy. Loss of FOXRED1, coupled with protein, choline and/or folate-deficient diets results in the depletion of glutathione, the dysregulation of nitric oxide metabolism and the peroxynitrite-mediated inactivation of complex I.
Keywords
ONOO-GSNOS-nitrosoglutathioneABCCPS1PEMTdihydrofolateADH3GSNORMDH2FTHFSDHFDTMPDimethylglycine dehydrogenaseFghIUGRsarcosine dehydrogenases-formylglutathione hydrolaseGSNO reductaseHMGSHDMGOETFALDHTHFGSHGSSGiNOSCFTRMitochondrial DNANADH:ubiquinone oxidoreductaseO2−ROSaldehyde dehydrogenasetetrahydrofolateThymidylatemtDNASuperoxide anion radicalcystic fibrosis transmembrane conductance regulatorSarcosine oxidaseinducible nitric oxide synthaseLeigh syndromeelectron transfer flavoproteinGlutathione metabolismintrauterine growth restrictionmalate dehydrogenaseNitric oxidePeroxynitriteATP-binding cassetteGlutathioneoxidized glutathioneReactive oxygen species
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Authors
Bernard D. Lemire,