ACADVL (P49748) variants and mutations

ACADVL (also known as P49748) is a human protein-coding gene encoding a very long-chain acyl-CoA dehydrogenase, mitochondrial protein. It catalyzes the first dehydrogenation step in mitochondrial beta-oxidation of long-chain fatty acids, especially during fasting and sustained energy demand. Biallelic loss of function causes very-long-chain acyl-CoA dehydrogenase deficiency, ranging from severe cardiomyopathy to exercise-induced rhabdomyolysis. This analysis covers 1,265 ACADVL variants and mutations. Of these, 80% have computational variant effect predictions. Disease context includes very long chain acyl-CoA dehydrogenase deficiency, hereditary disease, and long chain acyl-CoA dehydrogenase deficiency. Example ACADVL variants include M1I, Q2*, and Q2L.

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 ACADVL variants

Examples include M1I, Q2*, Q2L, Q2R, Q2H, A3T, A3V, A3S. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.