Name

NDUFV2P1 Gene

NADH dehydrogenase (ubiquinone) flavoprotein 2 pseudogene 1

SDHA Gene

succinate dehydrogenase complex, subunit A, flavoprotein (Fp)

This gene encodes a major catalytic subunit of succinate-ubiquinone oxidoreductase, a complex of the mitochondrial respiratory chain. The complex is composed of four nuclear-encoded subunits and is localized in the mitochondrial inner membrane. Mutations in this gene have been associated with a form of mitochondrial respiratory chain deficiency known as Leigh Syndrome. A pseudogene has been identified on chromosome 3q29. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jun 2014]

NDUFV2 Gene

NADH dehydrogenase (ubiquinone) flavoprotein 2, 24kDa

The NADH-ubiquinone oxidoreductase complex (complex I) of the mitochondrial respiratory chain catalyzes the transfer of electrons from NADH to ubiquinone, and consists of at least 43 subunits. The complex is located in the inner mitochondrial membrane. This gene encodes the 24 kDa subunit of complex I, and is involved in electron transfer. Mutations in this gene are implicated in Parkinson's disease, bipolar disorder, schizophrenia, and have been found in one case of early onset hypertrophic cardiomyopathy and encephalopathy. A non-transcribed pseudogene of this locus is found on chromosome 19. [provided by RefSeq, Oct 2009]

ETFA Gene

electron-transfer-flavoprotein, alpha polypeptide

ETFA participates in catalyzing the initial step of the mitochondrial fatty acid beta-oxidation. It shuttles electrons between primary flavoprotein dehydrogenases and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. Defects in electron-transfer-flavoprotein have been implicated in type II glutaricaciduria in which multiple acyl-CoA dehydrogenase deficiencies result in large excretion of glutaric, lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

ETFB Gene

electron-transfer-flavoprotein, beta polypeptide

This gene encodes electron-transfer-flavoprotein, beta polypeptide, which shuttles electrons between primary flavoprotein dehydrogenases involved in mitochondrial fatty acid and amino acid catabolism and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. The gene deficiencies have been implicated in type II glutaricaciduria. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jul 2008]

LOC220729 Gene

succinate dehydrogenase complex, subunit A, flavoprotein (Fp) pseudogene

NDUFV3 Gene

NADH dehydrogenase (ubiquinone) flavoprotein 3, 10kDa

The protein encoded by this gene is one of at least forty-one subunits that make up the NADH-ubiquinone oxidoreductase complex. This complex is part of the mitochondrial respiratory chain and serves to catalyze the rotenone-sensitive oxidation of NADH and the reduction of ubiquinone. The encoded protein is one of three proteins found in the flavoprotein fraction of the complex. The specific function of the encoded protein is unknown. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

NDUFV1 Gene

NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa

The mitochondrial respiratory chain provides energy to cells via oxidative phosphorylation and consists of four membrane-bound electron-transporting protein complexes (I-IV) and an ATP synthase (complex V). This gene encodes a 51 kDa subunit of the NADH:ubiquinone oxidoreductase complex I; a large complex with at least 45 nuclear and mitochondrial encoded subunits that liberates electrons from NADH and channels them to ubiquinone. This subunit carries the NADH-binding site as well as flavin mononucleotide (FMN)- and Fe-S-biding sites. Defects in complex I are a common cause of mitochondrial dysfunction; a syndrome that occurs in approximately 1 in 10,000 live births. Mitochondrial complex I deficiency is linked to myopathies, encephalomyopathies, and neurodegenerative disorders such as Parkinson's disease and Leigh syndrome. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Oct 2009]

SDHAP2 Gene

succinate dehydrogenase complex, subunit A, flavoprotein pseudogene 2

SDHAP3 Gene

succinate dehydrogenase complex, subunit A, flavoprotein pseudogene 3

SDHAP1 Gene

succinate dehydrogenase complex, subunit A, flavoprotein pseudogene 1

ETFDH Gene

electron-transferring-flavoprotein dehydrogenase

This gene encodes a component of the electron-transfer system in mitochondria and is essential for electron transfer from a number of mitochondrial flavin-containing dehydrogenases to the main respiratory chain. Mutations in this gene are associated with glutaric acidemia. Alternatively spliced transcript variants that encode distinct isoforms have been observed. [provided by RefSeq, Aug 2013]

ETFRF1 Gene

electron transfer flavoprotein regulatory factor 1

Involved in respiratory electron transport chain. Located in mitochondrion. [provided by Alliance of Genome Resources, Mar 2025]

ETFBKMT Gene

electron transfer flavoprotein subunit beta lysine methyltransferase

Enables heat shock protein binding activity and protein-lysine N-methyltransferase activity. Involved in negative regulation of fatty acid beta-oxidation using acyl-CoA dehydrogenase. Located in mitochondrial matrix. Part of protein-containing complex. [provided by Alliance of Genome Resources, Mar 2025]

Flavoprotein-like Gene Set

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Flavoprotein-like protein domain from the InterPro Predicted Protein Domain Annotations dataset.

electron transfer flavoprotein complex Gene Set

From COMPARTMENTS Text-mining Protein Localization Evidence Scores

proteins co-occuring with the electron transfer flavoprotein complex cellular component in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores dataset.

mitochondrial electron transfer flavoprotein complex Gene Set

From COMPARTMENTS Text-mining Protein Localization Evidence Scores

proteins co-occuring with the mitochondrial electron transfer flavoprotein complex cellular component in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores dataset.

flavoprotein Gene Set

From GeneRIF Biological Term Annotations

genes co-occuring with the biological term flavoprotein in literature-supported statements describing functions of genes from the GeneRIF Biological Term Annotations dataset.

electron-transferring-flavoprotein dehydrogenase activity Gene Set

From GO Molecular Function Annotations 2015

genes performing the electron-transferring-flavoprotein dehydrogenase activity molecular function from the curated GO Molecular Function Annotations 2015 dataset.

oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen Gene Set

From GO Molecular Function Annotations 2015

genes performing the oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen molecular function from the curated GO Molecular Function Annotations 2015 dataset.

electron transfer flavoprotein-ubiquinone oxidoreductase defect Gene Set

From HPO Gene-Disease Associations

genes associated with the electron transfer flavoprotein-ubiquinone oxidoreductase defect phenotype by mapping known disease genes to disease phenotypes from the HPO Gene-Disease Associations dataset.

Flavoprotein pyridine nucleotide cytochrome reductase Gene Set

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Flavoprotein pyridine nucleotide cytochrome reductase protein domain from the InterPro Predicted Protein Domain Annotations dataset.

CO dehydrogenase flavoprotein, C-terminal Gene Set

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the CO dehydrogenase flavoprotein, C-terminal protein domain from the InterPro Predicted Protein Domain Annotations dataset.

Succinate dehydrogenase, flavoprotein subunit Gene Set

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Succinate dehydrogenase, flavoprotein subunit protein domain from the InterPro Predicted Protein Domain Annotations dataset.

Electron transfer flavoprotein, alpha subunit, C-terminal, conserved site Gene Set

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Electron transfer flavoprotein, alpha subunit, C-terminal, conserved site protein domain from the InterPro Predicted Protein Domain Annotations dataset.

Succinate dehydrogenase/fumarate reductase flavoprotein, catalytic domain Gene Set

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Succinate dehydrogenase/fumarate reductase flavoprotein, catalytic domain protein domain from the InterPro Predicted Protein Domain Annotations dataset.

Flavoprotein Gene Set

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Flavoprotein protein domain from the InterPro Predicted Protein Domain Annotations dataset.

Electron transfer flavoprotein-ubiquinone oxidoreductase Gene Set

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Electron transfer flavoprotein-ubiquinone oxidoreductase protein domain from the InterPro Predicted Protein Domain Annotations dataset.

Succinate dehydrogenase/fumarate reductase, flavoprotein subunit Gene Set

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Succinate dehydrogenase/fumarate reductase, flavoprotein subunit protein domain from the InterPro Predicted Protein Domain Annotations dataset.

Electron transfer flavoprotein, beta subunit Gene Set

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Electron transfer flavoprotein, beta subunit protein domain from the InterPro Predicted Protein Domain Annotations dataset.

DNA/pantothenate metabolism flavoprotein, C-terminal Gene Set

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the DNA/pantothenate metabolism flavoprotein, C-terminal protein domain from the InterPro Predicted Protein Domain Annotations dataset.

Electron transfer flavoprotein, alpha/beta-subunit, N-terminal Gene Set

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Electron transfer flavoprotein, alpha/beta-subunit, N-terminal protein domain from the InterPro Predicted Protein Domain Annotations dataset.

Electron transfer flavoprotein, alpha subunit, C-terminal Gene Set

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Electron transfer flavoprotein, alpha subunit, C-terminal protein domain from the InterPro Predicted Protein Domain Annotations dataset.

NADH-ubiquinone oxidoreductase flavoprotein 3 Gene Set

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the NADH-ubiquinone oxidoreductase flavoprotein 3 protein domain from the InterPro Predicted Protein Domain Annotations dataset.

Electron transfer flavoprotein, beta-subunit, conserved site Gene Set

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Electron transfer flavoprotein, beta-subunit, conserved site protein domain from the InterPro Predicted Protein Domain Annotations dataset.

Electron transfer flavoprotein, alpha subunit Gene Set

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Electron transfer flavoprotein, alpha subunit protein domain from the InterPro Predicted Protein Domain Annotations dataset.

Electron Transfer Flavoprotein-Ubiquinone Oxidoreductase Defect Gene Set

From DisGeNET Gene-Phenotype Associations

genes associated with the phenotype Electron Transfer Flavoprotein-Ubiquinone Oxidoreductase Defect in GWAS and other genetic association datasets from the DisGeNET Gene-Phenotype Associations dataset.

oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen Gene Set

From GO Molecular Function Annotations 2023

genes performing the oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen molecular function from the curated GO Molecular Function Annotations 2023 dataset.

electron-transferring-flavoprotein dehydrogenase activity Gene Set

From GO Molecular Function Annotations 2023

genes performing the electron-transferring-flavoprotein dehydrogenase activity molecular function from the curated GO Molecular Function Annotations 2023 dataset.

oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen Gene Set

From GO Molecular Function Annotations 2025

genes performing the oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen molecular function from the curated GO Molecular Function Annotations 2025 dataset.

Electron transfer flavoprotein complex Gene Set

From COMPARTMENTS Curated Protein Localization Evidence Scores 2025

proteins localized to the Electron transfer flavoprotein complex cellular component from the COMPARTMENTS Curated Protein Localization Evidence Scores 2025 dataset.

Electron transfer flavoprotein complex Gene Set

From COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025

proteins co-occuring with the Electron transfer flavoprotein complex cellular component in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025 dataset.

Mitochondrial electron transfer flavoprotein complex Gene Set

From COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025

proteins co-occuring with the Mitochondrial electron transfer flavoprotein complex cellular component in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025 dataset.