ACAD9 (Q9H845) variants and mutations

ACAD9 (also known as Q9H845) is a human protein-coding gene encoding a complex I assembly factor ACAD9, mitochondrial protein. It supports mitochondrial energy production through long-chain fatty-acid oxidation and also functions as an assembly factor for respiratory-chain complex I. Biallelic pathogenic variants can cause complex I deficiency, cardiomyopathy, exercise intolerance, and other mitochondrial disease manifestations. This analysis covers 967 ACAD9 variants and mutations. Of these, 78% have computational variant effect predictions. Disease context includes acyl-CoA dehydrogenase 9 deficiency, mitochondrial complex I deficiency, and neurodegenerative disease. Example ACAD9 variants include M1I, M1L, and M1V.

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, Protein Data Bank, AlphaFold DB, gnomAD constraint, Open Targets, MaveDB, LitVar.

Notable ACAD9 variants

Examples include M1I, M1L, M1V, S2G, S2I, S2N, S2R, S2S. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.