ACADM (P11310) variants and mutations

ACADM (also known as P11310) is a human protein-coding gene encoding a medium-chain specific acyl-CoA dehydrogenase, mitochondrial protein. It performs the first oxidation step for medium-chain fatty acids during mitochondrial beta-oxidation, becoming especially important during fasting. Biallelic loss-of-function variants cause MCAD deficiency, with risk of hypoketotic hypoglycemia and sudden metabolic decompensation. This analysis covers 806 ACADM variants and mutations. Of these, 73% have computational variant effect predictions. Disease context includes medium chain acyl-CoA dehydrogenase deficiency, hereditary disease, and rhabdomyolysis. Example ACADM variants include M1?, M1I, and M1L.

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, ClinGen, MaveDB, LitVar.

Notable ACADM variants

Examples include M1?, M1I, M1L, M1R, M1V, A2V, A2T, A2E. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.