Beta-ketothiolase

Beta-ketothiolase (also known as Acetoacetyl-CoA thiolase) is an enzyme that plays a significant role in the metabolism of fatty acids and amino acids. It is involved in the ketone body and isoleucine catabolic pathways.

Function

Beta-ketothiolase specifically catalyzes the third step of ketone body degradation, which is the CoA-mediated thiolytic cleavage of acetoacetyl-CoA to yield 2 molecules of acetyl-CoA. In addition, it is also involved in the degradation of isoleucine and the anti catabolism of 2-methylacetoacetyl-CoA.

Clinical significance

Deficiency of this enzyme, an autosomal recessive inherited disorder, results in beta-ketothiolase deficiency, a metabolic disorder that can lead to metabolic acidosis, ketosis, and episodic encephalopathy.

See also

• Thiolase
• Acetoacetyl CoA
• Ketone bodies
• Metabolic pathway
• Metabolic disorder

External links

• GeneCards: ACAT1

Beta-ketothiolase Resources
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