Xaa-Pro dipeptidase, also known as prolidase, is an enzyme that in humans is encoded by the PEPDgene.
Xaa-Pro dipeptidase is a cytosolic dipeptidase that hydrolyzes dipeptides with proline or hydroxyproline at the carboxy terminus (but not Pro-Pro). It is important in collagen metabolism because of the high levels of imino acids. Mutations at the PEPD locus cause prolidase deficiency. This is characterised by Iminodipeptidurea, Skin ulcers, mental retardation and recurrent infections.
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