2-methylacetoacetyl-CoA thiolase

2-meth·yl·a·ce·to·a·ce·tyl-CoA thi·o·lase

(meth'il-a-sē'tō-a'sĕ-til thī'ō-lās),
An enzyme that is part of the l-isoleucine degradation pathway; it catalyzes the conversion of 2-methylacetoacetyl-CoA to acetyl-CoA and propionyl-CoA. A deficiency of this enzyme leads to an accumulation of 2-methylacetoacetyl-CoA, causing episodes of severe metabolic acidosis and ketosis.

thiolase

An obsolete generic term for any enzyme (e.g., acetyl CoA acetyltransferase [EC 2.3.1.9]) which catalyses a reaction between a compound, R–R’, and a thiol, R’–SH, forming R–S–R’ and R'–H.