Fig. 1From: 2-methylacetoacetyl-coenzyme A thiolase (beta-ketothiolase) deficiency: one disease - two pathways2-methylacetoacetyl-coenzyme A thiolase (MAT) is involved in both ketone body metabolism (top) and isoleucine catabolism (bottom). Metabolites which accumulate in MAT deficiency and can contribute to the diagnosis via abnormalities in urinary organic acids and blood acylcarnitines are printed in light blueBack to article page