Tiglyl carnitine plays a significant role in metabolic processes, particularly in the breakdown of certain amino acids and fatty acids. It is involved in the transport of tiglyl-CoA, a derivative of isoleucine metabolism, into the mitochondria for energy production. This compound is often studied in the context of metabolic disorders, as its accumulation can indicate disruptions in mitochondrial function or specific enzyme deficiencies. Clinically, measuring tiglyl carnitine levels is useful for diagnosing conditions like 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency or other inborn errors of metabolism. Additionally, it serves as a biomarker in newborn screening programs to identify metabolic abnormalities early in life. Research into tiglyl carnitine also contributes to understanding metabolic pathways and developing targeted therapies for related disorders.