Thiamine metabolic pathways (Homo sapiens)

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ArcPathVisio Brace Ellipse EndoplasmicReticulum GolgiApparatus HexagonPathVisio MimDegradation Mitochondria Octagon PentagonPathVisio Rectangle RoundedRectangle SarcoplasmicReticulum TriangleEquilateralEast TrianglePathVisio none MitochondrionPentose phosphate pathwayCitric acid cycleCytoplasmBranched chain acylCoAsThiamine monophosphateDNAGlucose 6-phosphateisocitrateGlucose 1-phosphatesuccinateTHTR1(SLC19A2)DHAPBranched chain amino acidsGlycerol 3-phosphateOxaloacetatecitratepyruvateD-Glyceraldehyde 3-phosphatethiamineThiamine pyrophosphate(TPP)serineTHTR2(SLC19A3)SLC25A19Acetyl-CoABCKDKalpha-ketoglutarateD-Ribose 5-phosphateTPK1TKTTPPPyruvate dehydrogenase E1TPPBranched chain keto acid dehydrogenase E1TPPOGDHTPPTPPsphingolipidsplasmalogensphospholipidsName: Thiamine metabolic pathwaysOrganism: Homo sapiens


Description

Thiamine enters the cell through THTRI and THTR2 receptors encoded by SLCI9A2 and SLC19A3 genes, respectively. It is then converted into thiamine pyrophosphate (TPP) by the thiamine phosphokinase enzyme encoded by TPK1 (5). TPP enters the mitochondria through the TPP transporter (TPP tr) encoded by the SLC25A19 gene. In the mitochondria, TPP serves as a cofactor for ketoglutarate-(2),pyruvate-(1) and branched-chain a-keto acid (3) dehydrogenases that are involved in the mitochondrial energy production from carbohydrates and amino acids. In the cytosol, TPP is a cofactor of the transketolase (4) in the pentose phosphate pathway. This pathway leads to the production of NAPDH, an important cofactor in biosynthetic pathways, as well as important intermediary molecules such as ribose 5-phosphate needed for the synthesis of nucleic acids and GA3P for the synthesis of complex lipids including plasmalogens, sphingolipids, and phospholipids. (Adapted from Lamari et al. (2013)). (Blau, CH15)

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Quality Tags

Image:Curated.pngReview changes for Approved
Image:Wplogo_31.pngCommunity: Rare Diseases
Image:Wplogo_31.pngCommunity: Inborn Errors of Metabolism (IEM)

Ontology Terms

 

Bibliography

  1. Rindi G, Laforenza U; ''Thiamine intestinal transport and related issues: recent aspects.''; Proc Soc Exp Biol Med, 2000 PubMed Europe PMC Scholia
  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

History

View all...
CompareRevisionActionTimeUserComment
129257
Approved
RARE
IEM
view11:17, 16 March 2024EgonwNot a mim-conversion
124272view12:41, 6 October 2022DeSlFixed interaction to label as Graphical Lines
124271view12:40, 6 October 2022DeSlAdded missing IDs
120409view09:26, 30 November 2021Fehrhartboxed pathway nodes
119258view19:46, 22 June 2021Finterlyfixed ISBN from ebook to hardcover
119253view19:37, 22 June 2021FinterlyAdded ISBN for book citation
110714view14:56, 28 May 2020Fehrhartadded type for plasmalogens (no ID available yet)
106795view13:27, 17 September 2019MaintBotHMDB identifier normalization
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

DataNodes

View all...
Name  ↓Type  ↓Database reference  ↓Comment  ↓
Acetyl-CoAMetaboliteCHEBI:15351 (ChEBI)
BCKDKGeneProductENSG00000103507 (Ensembl)
Branched chain acylCoAsMetabolite
Branched chain amino acidsMetaboliteCHEBI:22918 (ChEBI)
Branched chain keto acid dehydrogenase E1ProteinA0A024R0K3 (Uniprot-TrEMBL)
D-Glyceraldehyde 3-phosphateMetaboliteHMDB01112 (HMDB)
D-Ribose 5-phosphateMetaboliteHMDB01548 (HMDB)
DHAPMetaboliteCHEBI:16108 (ChEBI)
DNA
Glucose 1-phosphateMetaboliteHMDB01586 (HMDB)
Glucose 6-phosphateMetaboliteHMDB01401 (HMDB)
Glycerol 3-phosphateMetaboliteHMDB00126 (HMDB)
OGDHGeneProductENSG00000105953 (Ensembl)
OxaloacetateMetaboliteCHEBI:30744 (ChEBI)
Pyruvate dehydrogenase E1GeneProductENSG00000131828 (Ensembl)
SLC25A19GeneProductENSG00000125454 (Ensembl)
THTR1 (SLC19A2)GeneProductENSG00000117479 (Ensembl)
THTR2 (SLC19A3)GeneProductENSG00000135917 (Ensembl)
TKTGeneProductENSG00000163931 (Ensembl)
TPK1GeneProductENSG00000196511 (Ensembl)
TPPMetaboliteCHEBI:45931 (ChEBI)
Thiamine monophosphateMetaboliteCHEBI:9533 (ChEBI)
Thiamine pyrophosphate (TPP)MetaboliteCHEBI:45931 (ChEBI)
alpha-ketoglutarateMetaboliteCHEBI:16810 (ChEBI)
citrateMetaboliteCHEBI:133748 (ChEBI)
isocitrateMetaboliteCHEBI:16087 (ChEBI)
phospholipids
plasmalogens
pyruvateMetaboliteCHEBI:15361 (ChEBI)
serineMetaboliteCHEBI:17822 (ChEBI)
sphingolipids
succinateMetaboliteCHEBI:30031 (ChEBI)
thiamineMetaboliteCHEBI:33283 (ChEBI)

Annotated Interactions

No annotated interactions

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