Prolidase
Prolidase (also known as peptidase D or PEPD) is an enzyme that in humans is encoded by the PEPD gene. This enzyme is part of the protease family, specifically those acting on peptide bonds (peptidases). Prolidase has an important role in the metabolism of proline-containing di-peptides and is essential for the recycling of proline for subsequent protein synthesis.
Function[edit | edit source]
Prolidase is a cytosolic enzyme that catalyzes the hydrolysis of -Xaa-Pro dipeptides to Xaa and Pro. It plays an important role in the recycling of proline from degraded collagen and is also involved in the cellular immune response.
Clinical significance[edit | edit source]
Mutations in the PEPD gene have been associated with Prolidase deficiency (PD), a rare autosomal recessive disorder characterized by ulcers and other skin lesions, mental retardation, and recurrent respiratory tract infections. PD is the only known human disorder of proline metabolism.
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Contributors: Prab R. Tumpati, MD