2-methylacetoacetyl-CoA thiolase

2-methylacetoacetyl-CoA thiolase: 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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α-methylacetoacetyl CoA thiolase — α meth·yl·ac·e·to·ac·e·tyl CoA thi·o·lase (meth″əl ə se″to asґə tēl ko aґ thiґo lās) an acetyl CoA C acyltransferase (β ketothiolase) specifically catalyzing the final step in isoleucine catabolism,… … Medical dictionary
Acetyl-CoA C-acetyltransferase — In enzymology, an acetyl CoA C acetyltransferase (EC number|2.3.1.9) is an enzyme that catalyzes the chemical reaction:2 acetyl CoA ightleftharpoons CoA + acetoacetyl CoAHence, this enzyme has one substrate, acetyl CoA, and two products, CoA and… … Wikipedia
acetyl-CoA C-acyltransferase — ac·e·tyl CoA C acyl·trans·fer·ase (asґə təl ko aґ a″səl transґfər ās) (as″ə tēlґko aґ a″səl transґfər ās) [EC 2.3.1.16] any of several enzymes of the transferase class that… … Medical dictionary
List of diseases (0-9) — The List of diseases starting with a non letter is part of the complete list of diseases. NOTOC *11 beta hydroxylase deficiency *10q Partial trisomy, rare (NIH) A rare disease (occurrence fewer than 1 case for 200,000) according to the public… … Wikipedia
α-methylacetoaceticaciduria — α meth·yl·ac·e·to·ace·tic·ac·id·uria (meth″əl ə se″to ə se″tik as″ĭ duґre ə) an autosomal recessive aminoacidopathy due to deficiency of α methylacetoacetyl CoA thiolase, the β… … Medical dictionary

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