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.fceserine hydroxymethyltransferase 1, cytosolicSerine racemaseserine hydroxymethyltransferase 2; mitochondrialaka 7,8-dihydrofolatebf0Interaction only depicted in Blau book.fce"PHDGH catalyzes the first reaction of de novo serine biosynthesis, producing 3-phosphohydroxypyruvate by NAD+-coupled oxidation of 3-phosphoglycerate (3PG). The PHGDH reaction is reversible and, under standard conditions, thermodynamically favors the direction from 3-phosphohydroxypyruvate to 3PG. In cells engaged in serine synthesis, the reaction is driven toward 3-phosphohydroxypyruvate due to its consumption by downstream pathway steps." [PMID:25406093]fb5PSPH deficiencyDOID:0050724Human Disease Ontologyserine metabolic pathwayPW:0001084Pathway OntologyPHGDH deficiencyDOID:0050722Human Disease OntologyPSAT deficiencyDOID:0050723Human Disease Ontologyinherited metabolic disorderDOID:655Human Disease Ontology3642403360ISBNPhysician's Guidee to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases'Springer ISBN 36424033602014Blau NDuran MGibson KMDionisi-Vici C15536068PubMedSerine racemase modulates intracellular D-serine levels through an alpha,beta-elimination activity.J Biol Chem2005Foltyn VNBendikov IDe Miranda JPanizzutti RDumin EShleper MLi PToney MDKartvelishvily EWolosker H25406093PubMedHuman phosphoglycerate dehydrogenase produces the oncometabolite D-2-hydroxyglutarate.ACS Chem Biol2015Fan JTeng XLiu LMattaini KRLooper REVander Heiden MGRabinowitz JD