Serine metabolism (WP4688)
Homo sapiens
This pathway shows disorders related to serine metabolism. Disorders resulting from an enzyme defect are highlighted in pink. This pathway was inspired by Chapter 5 (ed. 4) from the book of Blau (ISBN 3642403360 (978-3642403361)), Fig. 5.2.
Authors
Amy Kutmon , Denise Slenter , Kristina Hanspers , Yasmin Omar , Egon Willighagen , Eric Weitz , Friederike Ehrhart , and Finterly HuActivity
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Organisms
Homo sapiensCommunities
Inherited Metabolic Disorders (IMD) Pathways Lipids and LIPID MAPS Rare DiseasesAnnotations
Disease Ontology
PSPH deficiency PHGDH deficiency PSAT deficiency inherited metabolic disorderPathway Ontology
serine metabolic pathway| Label | Type | Compact URI | Comment |
|---|---|---|---|
| D-serine | Metabolite | chebi:35247 | |
| 3-Phosphoserine | Metabolite | chebi:57524 | |
| Mg2+ | Metabolite | chebi:18420 | |
| NAD+ | Metabolite | chebi:15846 | |
| (2R)-3-phosphoglycerate | Metabolite | chebi:58272 | |
| Phosphatidylserine | Metabolite | chebi:18303 | |
| PLP | Metabolite | chebi:18405 | |
| Glycine | Metabolite | chebi:57305 | |
| Methylene-THF | Metabolite | chebi:15636 | |
| pyruvate | Metabolite | chebi:15361 | |
| glucose | Metabolite | chebi:17234 | |
| H+ | Metabolite | chebi:15378 | |
| D-Glyceraldehyde 3-phosphate | Metabolite | chebi:29052 | |
| 3-Phosphohydroxypyruvate | Metabolite | chebi:18110 | |
| L-serine | Metabolite | chebi:33384 | |
| NADH | Metabolite | chebi:57945 | |
| glutamate | Metabolite | chebi:29985 | |
| 2-Ketoglutarate | Metabolite | chebi:16810 | |
| H2O | Metabolite | chebi:15377 | |
| Pi | Metabolite | chebi:43474 | |
| dTMP | Metabolite | chebi:63528 | |
| dUMP | Metabolite | chebi:246422 | |
| DHF | Metabolite | chebi:57451 | aka 7,8-dihydrofolate |
| THF | Metabolite | chebi:57453 | |
| sphingolipids | Metabolite | chebi:26739 | |
| ceramides | Metabolite | chebi:17761 | |
| PHGDH | Protein | uniprot:O43175 | |
| TYMS | Protein | uniprot:P04818 | |
| SHMT1 | Protein | uniprot:P34896 | serine hydroxymethyltransferase 1, cytosolic |
| SR | Protein | uniprot:Q9GZT4 | Serine racemase |
| PSAT1 | Protein | uniprot:Q9Y617 | |
| SHMT2 | Protein | uniprot:P34897 | serine hydroxymethyltransferase 2; mitochondrial |
| DHFR | Protein | uniprot:P00374 | |
| PSPH | Protein | uniprot:P78330 |
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
- Serine racemase modulates intracellular D-serine levels through an alpha,beta-elimination activity. Foltyn VN, Bendikov I, De Miranda J, Panizzutti R, Dumin E, Shleper M, et al. J Biol Chem. 2005 Jan 21;280(3):1754–63. PubMed Europe PMC Scholia
- Human phosphoglycerate dehydrogenase produces the oncometabolite D-2-hydroxyglutarate. Fan J, Teng X, Liu L, Mattaini KR, Looper RE, Vander Heiden MG, et al. ACS Chem Biol. 2015 Feb 20;10(2):510–6. PubMed Europe PMC Scholia