Molecular Classification
Enzyme
Disease Roles
Metabolic disordersMedium Chain 3-Ketoacyl-CoA Thiolase Deficiency (MCKAT deficiency)

3-ketoacyl-CoA thiolase Overview

3-ketoacyl-CoA thiolase (KAT) catalyzes the final step of the β-oxidation cycle, which is crucial for fatty acid degradation. It cleaves a two-carbon unit from 3-ketoacyl-CoA, producing acetyl-CoA and a shortened acyl-CoA. KAT can also catalyze the reverse Claisen condensation reaction between two acyl-CoAs, enabling carbon chain elongation. Deficiency can lead to metabolic disorders like MCKAT deficiency.

Mechanism of Action

Biological Functions

Fatty acid degradation
β-oxidation
Carbon chain elongation

Disease Associations

Metabolic disorders
Medium Chain 3-Ketoacyl-CoA Thiolase Deficiency (MCKAT deficiency)

Safety Considerations

  • Impaired breakdown of certain fats
  • Accumulation of toxic intermediates