Biochemistry of Proline and Hydroxyproline (Homo sapiens)

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1Several stepsinvolvedcollagenFADgamma-glutamyl-PprolineNADPP5CRX-OH-prolineglycyl-prolineASAOATP5COATPEPDPRODHglutamiccitrullineornithineP5CSP5CSglutamic-semialdehydeNADPHH+glutamateNAD+ALDH4A1NADPH+NH3arginineNADPH+NAD+FADH2alanyl-prolineX-prolineOH-prolinedelta1-pyrroline-3-hydroxy-5-carboxylatePRODHPyrroline-5-carboxylate synthase deficiencyHyper-prolinemia, Type IIHyper-prolinemia, Type IHyper-prolinemia, Type IHypo-prolinemia, Type IIBHypo-prolinemia, Type IIIBProlidase deficiencyDiseaseCatalysisPathwayStimulationProteinMetaboliteConversionConnection to diseaseLegend


Description

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.

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Ontology Terms

 

Bibliography

  1. Nenad Blau, Carlo Dionisi Vici, K Michael Gibson, Marinus Duran; ''Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases''; Springer (ISBN-13: 978-3642403361), 2014

History

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CompareRevisionActionTimeUserComment
114133view10:32, 23 December 2020DeSlUpdate description with fig. number.
114128view10:24, 23 December 2020DeSlOntology Term : 'prolidase deficiency pathway' added !
114127view10:24, 23 December 2020DeSlOntology Term : 'prolidase deficiency' added !
114126view10:24, 23 December 2020DeSlOntology Term : 'hyperprolinemia' added !
114125view10:24, 23 December 2020DeSlOntology Term : 'hyperprolinemia type 2' added !
114124view10:24, 23 December 2020DeSlOntology Term : 'hyperprolinemia type II pathway' added !
114123view10:24, 23 December 2020DeSlOntology Term : 'hyperprolinemia type I pathway' added !
114122view10:23, 23 December 2020DeSlOntology Term : 'hyperprolinemia type 1' added !
114121view10:23, 23 December 2020DeSlOntology Term : 'arginine and proline metabolic pathway' added !
114120view10:22, 23 December 2020DeSlOntology Term : 'proline metabolic pathway' added !
114119view10:22, 23 December 2020DeSlOntology Term : 'glutathione synthase deficiency pathway' added !
114090view07:28, 22 December 2020EgonwRemove RHEA: prefix in identifier
114062view15:01, 18 December 2020DeSlUpdated layout of disease interactions, added legend
114061view14:58, 18 December 2020DeSlUpdated description
114060view14:52, 18 December 2020DeSlAdded disorders (including OMIM ID); annotated some proteins
114059view14:34, 18 December 2020DeSlStarted adding IDs
114052view12:55, 18 December 2020DeSlAdded remaining steps
114049view12:44, 18 December 2020DeSlNew pathway

External references

DataNodes

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NameTypeDatabase referenceComment
ALDH4A1ProteinP30038 (Uniprot-TrEMBL) aka P5CDH
ASAMetaboliteCHEBI:57472 (ChEBI) aka argininosuccinic acid
FADMetabolite
FADH2Metabolite
H+Metabolite
NAD+Metabolite
NADPMetabolite
NADPHMetabolite
NH3Metabolite
OATProtein
OATProteinP04181 (Uniprot-TrEMBL)
OH-prolineMetabolite
P5CMetaboliteCHEBI:17388 (ChEBI) aka delta1-pyrroline-5-carboxylate
P5CRProteinP32322 (Uniprot-TrEMBL) aka delta-1-pyrroline-5-carboxylate reductase
P5CSProteinP30038 (Uniprot-TrEMBL) aka delta-1-pyrroline-5-carboxylate synthetase
PEPDProteinP12955 (Uniprot-TrEMBL)
PRODHProteinO43272 (Uniprot-TrEMBL) aka proline dehydrogenase
X-OH-prolineMetabolite
X-prolineMetabolite
alanyl-prolineMetabolite
arginineMetaboliteCHEBI:32682 (ChEBI)
citrullineMetaboliteCHEBI:57743 (ChEBI)
collagenProtein
delta1-pyrroline-3-hydroxy- 5-carboxylateMetabolite
gamma-glutamyl-PMetabolite
glutamateMetaboliteCHEBI:29985 (ChEBI)
glutamicMetabolite
glutamic-semialdehydeMetabolite
glycyl-prolineMetabolite
ornithineMetaboliteCHEBI:46911 (ChEBI)
prolineMetaboliteCHEBI:60039 (ChEBI)

Annotated Interactions

SourceTargetTypeDatabase referenceComment
P5Cprolinemim-conversion14107 (Rhea)
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