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Transketolase !

Transketolase swaps two-carbon units between sugars in the pentose pathway; it needs thiamine (vitamin B1).

Updated 2026-11-01

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Key facts

  • It moves 2 carbons from a ketose (xylulose 5-phosphate) to an aldose (ribose 5-phosphate or erythrose 4-phosphate).
  • Cofactor: thiamine pyrophosphate (TPP), the same as PDH and α-ketoglutarate dehydrogenase.
  • It links the pentose pathway with glycolysis in both directions (non-oxidative, reversible phase).
  • Structure 3MOS (human) has TPP in the active site.
EC number
2.2.1.1
Genes
TKT
Structure (PDB)
3MOS

Regulation

  • It depends on substrate availability and on thiamine.

Inhibitors and toxins

  • Oxythiamine (thiamine antagonist)

Clinical notes

  • Red cell transketolase activity, before and after adding TPP, measures thiamine deficiency.
  • A variant with low affinity for TPP has been linked to higher risk of Wernicke-Korsakoff syndrome in people with alcoholism.
  • Biallelic TKT variants cause short stature, developmental delay and congenital heart disease (very rare).

Pathways

Test yourself

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Which vitamin does transketolase need?

Thiamine (B1), as thiamine pyrophosphate.

How is transketolase used to measure thiamine deficiency?

Its activity is measured in red cells; if it rises a lot when TPP is added, the person was low in thiamine.

How many carbons does transketolase transfer and how many does transaldolase?

Transketolase transfers 2 and transaldolase 3.

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Educational information, not medical advice. For any symptom, see a health professional.

Sources: TKT: Transketolase (human) (UniProt, 2026); 3MOS: The structure of human Transketolase (RCSB Protein Data Bank, 2026); Pentose phosphate pathway (Reactome, 2026); Textbook of Biochemistry for Medical Students, 6th ed. (Jaypee Brothers Medical Publishers (Vasudevan DM, Sreekumari S, Vaidyanathan K), 2011).

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