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.
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An obsolete generic term for any enzyme (e.g., acetyl CoA acetyltransferase [EC]) which catalyses a reaction between a compound, R–R’, and a thiol, R’–SH, forming R–S–R’ and R'–H.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.
References in periodicals archive ?
B-ketothiolase (2-methylacetoacetyl-CoA thiolase) deficiency: A frequent disease in Tunisia?
2-EHA was first identified in the urine of a patient with [beta]-ketothiolase (2-methylacetoacetyl-CoA thiolase) deficiency (15).