Thiamine metabolic pathways (Homo sapiens)

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2MitochondrionPentose phosphate pathwayPlasma MembraneCitric acid cycleCytoplasmTHTR1 deficiencyDNASpingolipids pathwayGlucose 6-phosphateTPK1D-Ribose 5-phosphateserineBranched chain acylCoAsTHTR2(SLC19A3)OxaloacetateOGDHBranched chain keto acid dehydrogenase E1Thiamine pyrophosphate(TPP)Pyruvate dehydrogenase E1isocitratePhospholipidpathwayGlycerol 3-phosphateSLC25A19DHAPBCKDKD-Glyceraldehyde 3-phosphatepyruvateGlucose 1-phosphateAcetyl-CoAcitrateTHTR1(SLC19A2)TKTBranched chain amino acidsalpha-ketoglutaratesuccinatePPTPPTPPTPPTPPTPPsphingolipidsplasmalogensphospholipidsWernicke's-like encephalopathyand BRBGBilateral stiatalnecrosisThiamine monophosphate (TMP)1thiamineP


Thiamine is absorbed in the form of thiamine or thiamine monophosphate and transported through the plasma membrane by thiamine transporter 1 (THTR1) and thiamine transporter 2 (THTR2), which are encoded for by the genes SLC19A2 and SLC19A3, respectively. Upon cell entrance, thiamine undergoes pyrophosphorylation by thiamine pyrophosphokinase (TPK) to thiamine pyrophosphate (TPP). TPP is the active form of thiamine and serves as a cofactor of transketolase in the pentose phosphate pathway in the cytosol. TPP also functions as a cofactor for pyruvate, ketoglutarate and branched chain alpha-keto acid dehydrogenases in the mitochondrion after entrance via TPP transporter encoded by SLC25A19. This pathway was inspired by Chapter 15 (Thiamine Disorders) of the book of Blau (ISBN 3642403360 (978-3642403361)).

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Ontology Terms



  1. Rindi G, Laforenza U; ''Thiamine intestinal transport and related issues: recent aspects.''; Proc Soc Exp Biol Med, 2000 PubMed Europe PMC
  2. Blau, Nenad, Duran, Marinus, Gibson, K. Michael, Dionisi-Vici, Carlo; ''Physician's Guidee to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases''''; ISBN 978-3-642-40337-8 , Chapter 15, 2014


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104026view17:40, 25 April 2019IreneHemelModified description
98951view12:24, 17 October 2018DeSlModified description
98309view11:43, 20 August 2018DeSlModified description
98308view11:41, 20 August 2018DeSlChanged layout of disease nodes.
97959view14:04, 26 June 2018Youssefwalidadded lit ref for thiamine absorption
97958view13:18, 26 June 2018DeSlModified description
97957view13:13, 26 June 2018DeSlAdded phosphorylation states
97930view09:50, 25 June 2018Youssefwalidmade red lines thicker and corrected thiamine absorption nodes
97903view12:19, 22 June 2018YoussefwalidConnected lines
97846view08:28, 11 June 2018YoussefwalidModified description
97796view15:12, 8 June 2018EgonwReplaced a secondary ChEBI identifier with a primary identifier.
97795view13:33, 8 June 2018DeSlOntology Term : 'energy metabolic pathway' added !
97794view13:29, 8 June 2018DeSlMinor layout changes
97793view13:19, 8 June 2018DeSlAdded PW nodes+links, disease nodes+ URLs to OMIM
97792view13:04, 8 June 2018DeSlOntology Term : 'thiamine metabolic pathway' added !
97791view12:47, 8 June 2018DeSlAdded lit. ref.
97755view13:18, 7 June 2018YoussefwalidAdded node annotations
97609view08:40, 30 May 2018Youssefwalid
97585view06:36, 27 May 2018EgonwReplaced secondary ChEBI identifiers with primary identifiers.
97496view14:08, 23 May 2018Youssefwalid
97495view14:02, 23 May 2018YoussefwalidModified description
97493view14:00, 23 May 2018YoussefwalidNew pathway

External references


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NameTypeDatabase referenceComment
Acetyl-CoAMetaboliteCHEBI:15351 (ChEBI)
BCKDKGeneProductENSG00000103507 (Ensembl)
Branched chain

keto acid

dehydrogenase E1
ProteinA0A024R0K3 (Uniprot-TrEMBL)
Branched chain acylCoAsMetabolite
Branched chain amino acidsMetaboliteCHEBI:22918 (ChEBI)
Citric acid cyclePathway
D-Glyceraldehyde 3-phosphateMetaboliteHMDB01112 (HMDB)
D-Ribose 5-phosphateMetaboliteHMDB01548 (HMDB)
DHAPMetaboliteCHEBI:16108 (ChEBI)
DNAMetaboliteCHEBI:16991 (ChEBI)
Glucose 1-phosphateMetaboliteHMDB01586 (HMDB)
Glucose 6-phosphateMetaboliteHMDB01401 (HMDB)
Glycerol 3-phosphateMetaboliteHMDB00126 (HMDB)
OGDHGeneProductENSG00000105953 (Ensembl)
OxaloacetateMetaboliteCHEBI:30744 (ChEBI)


PathwayWP134 (WikiPathways)
Phospholipid pathwayPathwayWP3933 (WikiPathways)


GeneProductENSG00000131828 (Ensembl)
SLC25A19GeneProductENSG00000125454 (Ensembl)
Spingolipids pathwayPathwayWP4142 (WikiPathways)
THTR1 (SLC19A2)GeneProductENSG00000117479 (Ensembl)
THTR2 (SLC19A3)GeneProductENSG00000135917 (Ensembl)
TKTGeneProductENSG00000163931 (Ensembl)
TPK1GeneProductENSG00000196511 (Ensembl)
TPPMetaboliteCHEBI:45931 (ChEBI)
Thiamine monophosphate (TMP)MetaboliteCHEBI:9533 (ChEBI) Thiamine enters the cell via diffusion or transphosphorylation to thiamine monophosphate (TMP).
Thiamine pyrophosphate (TPP)MetaboliteCHEBI:45931 (ChEBI)
alpha-ketoglutarateMetaboliteCHEBI:16810 (ChEBI)
citrateMetaboliteCHEBI:133748 (ChEBI)
isocitrateMetaboliteCHEBI:16087 (ChEBI)
phospholipidsMetaboliteCHEBI:16247 (ChEBI)
pyruvateMetaboliteCHEBI:15361 (ChEBI)
serineMetaboliteCHEBI:17822 (ChEBI)
sphingolipidsMetaboliteCHEBI:26739 (ChEBI)
succinateMetaboliteCHEBI:30031 (ChEBI)
thiamineMetaboliteCHEBI:33283 (ChEBI)

Annotated Interactions

No annotated interactions

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