L-aminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferase (AASDHPPT)

L-aminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferase (AASDHPPT) is an essential enzyme that catalyzes the transfer of the 4′-phosphopantetheine group from coenzyme A to specific serine residues on acyl carrier proteins and related domains in target proteins. This post-translational modification, termed phosphopantetheinylation, is critical for the activation of enzymes in fatty acid synthesis, lysine metabolism, and folate metabolism. In mammals, only a single AASDHPPT gene encodes the unique phosphopantetheinyl transferase responsible for these modifications, localizing to both the cytoplasm and mitochondria (the latter via a mitochondrial targeting sequence). Deficiency or loss of AASDHPPT function is associated with metabolic disorders such as pipecolic acidemia and impaired mitochondrial fatty acid synthesis, leading to defects in oxidative metabolism and broader metabolic dysregulation in affected tissues. To date, direct pharmacological targeting strategies for this enzyme have not been clinically described; however, its central regulatory role positions it as a key point of control in related metabolic pathways.

Other names

AASDHPPTAminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferaseLYS5LYS2AASD-PPTCGI-80ACPSAlpha-aminoadipic semialdehyde dehydrogenase-phosphopantetheinyl transferaseholo ACP synthaseholo-[acyl-carrier-protein] synthase

For pantothenate: increases supply of CoA, supporting function and correcting deficiencies in phosphopantetheinylation General mechanisms: not targeted by approved drugs as a direct protein target