CPT2 (P23786) variants and mutations

CPT2 (also known as P23786) is a human protein-coding gene encoding a carnitine O-palmitoyltransferase 2, mitochondrial protein. It converts long-chain acylcarnitines back to acyl-CoA inside mitochondria, allowing long-chain fatty acids to undergo beta-oxidation. Biallelic deficiency causes a spectrum from lethal neonatal disease to recurrent exercise- or fasting-triggered rhabdomyolysis. This analysis covers 1,052 CPT2 variants and mutations. Of these, 82% have computational variant effect predictions. Disease context includes carnitine palmitoyl transferase II deficiency, myopathic form, carnitine palmitoyl transferase II deficiency, severe infantile form, and carnitine palmitoyl transferase II deficiency, neonatal form. Example CPT2 variants include V2E, V2L, and V2M.

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, MaveDB, LitVar.

Notable CPT2 variants

Examples include V2E, V2L, V2M, V2A, V2V, P3L, P3S, P3T. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.