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Thiamine-Dependent Enzyme

Molecular classification
Enzyme
01

Overview

Thiamine-dependent enzymes are a class of enzymes that require thiamine (vitamin B1), specifically its active coenzyme form thiamine diphosphate (TDP, also known as thiamine pyrophosphate or TPP), to catalyze key metabolic reactions. These enzymes play essential roles in energy production, amino acid metabolism, and biosynthetic pathways across all domains of life. Key enzymes include pyruvate dehydrogenase complex (PDH), α-Ketoglutarate dehydrogenase complex (KGDHC), transketolase (TKT), and branched-chain α-ketoacid dehydrogenase complex (BCKDH). Deficiency leads to impaired energy metabolism, neurological symptoms, lactic acidosis, and diseases such as beriberi or Wernicke-Korsakoff syndrome in humans.

02

Mechanism of action

Inhibition of enzyme activity through thiamine depletion or competitive inhibition.

03

Biological functions

Energy metabolismPentose phosphate pathwayAmino acid catabolism
04

Disease associations

Metabolic disordersNeurodegenerative disease
05

Safety considerations

Neurological symptomsLactic acidosis

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