Proline and hydroxyproline pathways (WP5026)
Homo sapiens
There are three disease tyes affecting proine metabolism. 1. Affecting catabolism of proline (e.g. hyperprolinemia type I and type II); 2. Affecting synthesis of proline (e.g. P5CS- and P5CR-deficiencies); 3. Accumulation of proline-containing peptides (e.g. prolidase deficiency). This pathway was inspired by Chapter 5 (edition 4) of the book of Blau (ISBN 3642403360 (978-3642403361)), Fig. 5.4.
Authors
Denise Slenter , Egon Willighagen , Eric Weitz , Finterly Hu , and Friederike EhrhartActivity
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Organisms
Homo sapiensCommunities
Rare DiseasesAnnotations
Disease Ontology
hyperprolinemia type 2 hyperprolinemia prolidase deficiency hyperprolinemia type 1Pathway Ontology
prolidase deficiency pathway hyperprolinemia type II pathway proline metabolic pathway arginine and proline metabolic pathway glutathione synthase deficiency pathway hyperprolinemia type I pathway| Label | Type | Compact URI | Comment |
|---|---|---|---|
| P5C | Metabolite | chebi:17388 | aka delta1-pyrroline-5-carboxylate |
| Citrulline | Metabolite | chebi:57743 | |
| Proline | Metabolite | chebi:60039 | |
| Gamma-glutamyl-P | Metabolite | chebi:50164 | |
| Glycyl-proline | Metabolite | pubchem.compound:3013625 | |
| FAD | Metabolite | chebi:16238 | |
| Ornithine | Metabolite | chebi:46911 | |
| ASA | Metabolite | chebi:57472 | aka argininosuccinic acid |
| NADP | Metabolite | chebi:25523 | |
| NADPH | Metabolite | chebi:16474 | |
| H+ | Metabolite | chebi:15378 | |
| Glutamate | Metabolite | chebi:29985 | |
| NAD+ | Metabolite | chebi:15846 | |
| NH3 | Metabolite | chebi:16134 | |
| Arginine | Metabolite | chebi:32682 | |
| FADH2 | Metabolite | chebi:17877 | |
| Alanyl-proline | Metabolite | pubchem.compound:83525 | |
| Delta1-pyrroline-3-hydroxy-5-carboxylate | Metabolite | pubchem.compound:23615442 | |
| P5CS | Protein | uniprot:P30038 | aka delta-1-pyrroline-5-carboxylate synthetase |
| PEPD | Protein | uniprot:P12955 | |
| PRODH | Protein | uniprot:O43272 | aka proline dehydrogenase |
| OAT | Protein | uniprot:P04181 | |
| Collagen | Protein | wikidata:Q26868 | |
| P5CR | Protein | uniprot:P32322 | aka delta-1-pyrroline-5-carboxylate reductase |
| ALDH4A1 | Protein | uniprot:P30038 | aka P5CDH |
References
- Physician’s Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases [Internet]. Blau N, Duran M, Gibson KM, Dionisi-Vici C. Springer; 2014. 0 p. Available from: https://books.google.com/books/about/Physician_s_Guide_to_the_Diagnosis_Treat.html?hl=&id=wJRBnwEACAAJ OpenLibrary Worldcat