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. fce serine hydroxymethyltransferase 1, cytosolic Serine racemase serine hydroxymethyltransferase 2; mitochondrial aka 7,8-dihydrofolate bf0 Interaction 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] fb5 PSPH deficiency DOID:0050724 Human Disease Ontology serine metabolic pathway PW:0001084 Pathway Ontology PHGDH deficiency DOID:0050722 Human Disease Ontology PSAT deficiency DOID:0050723 Human Disease Ontology inherited metabolic disorder DOID:655 Human Disease Ontology 3642403360 ISBN Physician's Guidee to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases' Springer ISBN 3642403360 2014 Blau N Duran M Gibson KM Dionisi-Vici C 15536068 PubMed Serine racemase modulates intracellular D-serine levels through an alpha,beta-elimination activity. J Biol Chem 2005 Foltyn VN Bendikov I De Miranda J Panizzutti R Dumin E Shleper M Li P Toney MD Kartvelishvily E Wolosker H 25406093 PubMed Human phosphoglycerate dehydrogenase produces the oncometabolite D-2-hydroxyglutarate. ACS Chem Biol 2015 Fan J Teng X Liu L Mattaini KR Looper RE Vander Heiden MG Rabinowitz JD