PYCS: Human pyrroline-5-carboxylate synthetase 1 - aldehyde dehydrogenase domain
PDB Code: 2H5G
The synthesis of the amino acids proline, ornithine and arginine is initiated by a two step process consisting of phosphorylation and an NADPH-dependent reduction of the phosphorylated intermediate, by using glutamate or N-acetyl glutamate as initial substrates.
PYCS is involved in the initial steps of proline synthesis, and is a bifunctional enzyme with two distinct enzyme domains. The kinase domain, which is located at the N-terminus of PYCS, catalyzes the phosphorylation of glutamate to γ-glutamyl phosphate. This intermediate is then reduced to the glutamate-γ-semialdehyde product by the C-terminal oxidoreductase domain of PYCS. The resulting semialdehyde product of this reaction spontaneously cyclizes to form pyrroline-5-carboxylate, which is reduced to proline by a subsequent enzymatic step carried out by the distinct enzyme pyrroline carboxylate reductase.
Mutations in PYCS have been thus far identified only in the kinase domain, and lead to hyperammonemia and deficiencies in ornithine, citrullin, arginine and proline levels, and may be associated with neurodegeneration, cataracts and diseases of connective tissues.
The oxidoreductase domain of PYCS is an atypical member of the aldehyde dehydrogenase (ALDH) superfamily, and is classified as ALDH18A1.

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