Proline and hydroxyproline pathways (WP5026)

Homo sapiens

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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 Ehrhart

Activity

last edited

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Organisms

Homo sapiens

Communities

Inherited Metabolic Disorders (IMD) Pathways Rare Diseases

Annotations

Disease Ontology

hyperprolinemia type 2 hyperprolinemia prolidase deficiency hyperprolinemia type 1

Pathway Ontology

prolidase deficiency pathway hyperprolinemia type II pathway proline metabolic pathway arginine and proline metabolic pathway glutathione synthase deficiency pathway hyperprolinemia type I pathway

Participants
Query Drugst.One

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

  1. 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