Very long-chain acyl-CoA dehydrogenase (VLCAD)

Very long-chain acyl-CoA dehydrogenase (VLCAD) is an enzyme located in the inner mitochondrial membrane that catalyzes the first and rate-limiting step of mitochondrial β-oxidation of very long-chain fatty acids (typically C14–C24). It is crucial for converting fat into usable energy, particularly during fasting or periods of increased energy demand. VLCAD deficiency, caused by mutations in the ACADVL gene, impairs this metabolic pathway and can result in life-threatening episodes of hypoglycemia, cardiomyopathy, liver dysfunction, and muscle weakness. The condition is inherited in an autosomal recessive manner and is screened in newborns. Treatment includes avoidance of fasting, dietary fat modifications (including supplementation with medium chain triglycerides), and other supportive measures. VLCAD is a validated therapeutic target due to its critical role in fatty acid metabolism and its direct linkage to human disease

Other names

Very long chain acyl-CoA dehydrogenaseVery-long chain acyl-CoA dehydrogenaseVery long chain acylCoA dehydrogenaseVLCADACADVLlong chain fatty acid oxidation disordernonketotic hypoglycemia caused by deficiency of acyl-CoA dehydrogenase

MCT oil supplies fats that bypass VLCAD and are oxidizable through alternative metabolic pathways. L-carnitine increases fatty acid transport and may promote residual β-oxidation.