Biochemistry · Enzyme
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.
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
Related conditions
Related
- Ribose 5-phosphate
- Glucose-6-phosphate dehydrogenase (G6PD)
- Pyruvate dehydrogenase complex (PDH)
- α-Ketoglutarate dehydrogenase
- NADPH
Test yourself
Think of the answer, then tap the question to see it.
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.
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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Representative model, for educational purposes only.