ETFDH (Q16134) variants and mutations

ETFDH (also known as Q16134) is a human protein-coding gene encoding an electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial protein. It transfers electrons from electron-transfer flavoprotein to ubiquinone in the inner mitochondrial membrane, linking several dehydrogenases to the respiratory chain. Biallelic deficiency causes multiple acyl-CoA dehydrogenase deficiency, often with a riboflavin-responsive late-onset myopathic form. This analysis covers 1,051 ETFDH variants and mutations. Of these, 80% have computational variant effect predictions. Disease context includes multiple acyl-CoA dehydrogenase deficiency, glutaric acidemia IIc, and glutaric aciduria. Example ETFDH variants include M1I, M1R, and M1T.

Variant analysis overview

Variant and mutation evidence

Clinical, disease, and population context

Protein structure and variant hotspots

Data sources

Evidence in this analysis draws on EBI Proteins Variation, UniProt, gnomAD v4, EuropePMC, 3D Hotspot Analysis, Interaction Network Analysis, AlphaFold DB, gnomAD constraint, Open Targets, ClinGen, MaveDB, LitVar.

Notable ETFDH variants

Examples include M1I, M1R, M1T, L2P, L2M, L2V, L2L, V3L. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.