AASDHPPT


L-aminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferase is an enzyme that in humans is encoded by the AASDHPPT gene. The AASDHPPT protein catalyzes the post-translational modification phosphopantetheinylation, in which a 4'-phosphopantetheine group derived from coenzyme A is covalently attached to target proteins in both cytosol and mitochondria. In the cytosol, these include fatty acid synthase, aminoadipate semialdehyde dehydrogenase, and aldehyde dehydrogenase 1 [family member L1], whereas in mitochondria the targets are the mitochondrial acyl carrier protein, and dehydrogenase/reductase 2. It has been suggested that defects in the human gene result in pipecolic acidemia.
The protein encoded by this gene is similar to Saccharomyces cerevisiae LYS5, which is required for the activation of the alpha-aminoadipate dehydrogenase in the biosynthetic [pathway of lysine]. Yeast alpha-aminoadipate dehydrogenase converts alpha-biosynthetic-aminoadipate semialdehyde to alpha-aminoadipate.