Isovaleryl-CoA dehydrogenase is an enzyme that plays a crucial role in the breakdown of certain fatty acids and amino acids. It is involved in the metabolism of isovaleryl-CoA, a compound derived from the amino acid leucine. This enzyme helps convert isovaleryl-CoA into another molecule, facilitating energy production and the proper functioning of cellular processes. Deficiencies in isovaleryl-CoA dehydrogenase can lead to a metabolic disorder known as isovaleric acidemia. This condition results in the accumulation of isovaleryl-CoA and its byproducts, which can be toxic and cause various health issues. Early diagnosis and management are essential to prevent complications associated with this disorder.