Accession NumberHMDB06695
Common_NameProlylhydroxyproline
DescriptionProlylhydroxyproline is a dipeptide. Prolylhydroxyproline is a marker of bone collagen degradation, showing high sensitivity for the diagnosis of osteoporosis. Prolylhydroxyproline has been suggested as a possible alternative to hydroxyproline determination in bone resorption studies. Prolylhydroxyproline is one of the iminodipeptides present in the urine of patients with prolidase deficiency. Prolidase (X-Pro dipeptidase EC 3.4. 13.9) splits iminodipeptides containing C-terminal proline or hydroxyproline (X-Pro or X-Hyp) to X+Pro or X+Hyp. Prolidase deficiency is a rare autosomal recessive disease characterized by chronic ulcerative dermatitis and mental retardation. These patients excrete large amounts of iminodipeptides containing C-terminal proline in the urine due to hereditary prolidase deficiency. (PMID: 12636053, 11863289, 2387877, 1874885, 9586797)
Chemical_IUPAC_Name(2S,4R)-4-hydroxy-1-[(2S)-pyrrolidine-2-carbonyl]pyrrolidine-2-carboxylic acid
Chemical FormulaC10H16N2O4
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