PEPD 抗体 (47-Q): sc-100708

PEPD (peptidase D), also referred to as prolidase, is a cytosolic dipeptidase that belongs to the peptidase M24B family. PEPD hydrolyzes di- and tripeptides with proline or hydroxyproline at the C-terminus. PEPD functions as a homodimer and may play an important role in collagen metabolism as well as in the recycling of proline in various cells and tissues. Defects in the gene encoding PEPD are the primary cause of prolidase deficiency in humans. Prolidase deficiency is an autosomal recessive disorder associated with iminodipeptiduria and is characterized by skin ulcers, mental retardation, recurrent infections and A-typical facies. Mutations in the gene encoding PEPD may also be the cause of systemic lupus erythematosus and necrosis-like cell death in fibroblasts. Additionally, there is thought to be a tight linkage between the polymorphisms of prolidase and the myotonic dystrophy trait.