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)).

Authors

Youssef Walid , Egon Willighagen , Denise Slenter , Irene Hemel , Friederike Ehrhart , and Finterly Hu

Cited In

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Organism

Homo sapiens

Communities

Inborn Errors of Metabolism (IEM) Pathways Rare Diseases

Annotations

Pathway Ontology: energy metabolic pathway thiamine metabolic pathway

Participants

References

1. Blau N, Duran M, Gibson KM, Dionisi-Vici C. Physician’s Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases [Internet]. Springer; 2014. 867 p. Available from: https://books.google.com/books/about/Physician_s_Guide_to_the_Diagnosis_Treat.html?hl=&id=wJRBnwEACAAJ OpenLibrary Worldcat
2. Rindi G, Laforenza U. Thiamine intestinal transport and related issues: recent aspects. Proc Soc Exp Biol Med. 2000 Sep;224(4):246–55. PubMed Europe PMC Scholia