Article
Crystal structure of human 3-hydroxy-3-methylglutaryl-CoA Lyase: insights into catalysis and the molecular basis for hydroxymethylglutaric aciduria.
The Journal of biological chemistry - 17 Mar 2006
Fu Zhuji, Runquist Jennifer A, Forouhar Farhad, Hussain Munif, Hunt John F, Miziorko Henry M, Kim Jung-Ja P
Abstract excerpt
3-Hydroxy-3-methylglutaryl-CoA (HMG-CoA) lyase is a key enzyme in the ketogenic pathway that supplies metabolic fuel to extrahepatic tissues. Enzyme deficiency may be due to a variety of human mutations and can be fatal. Diminished activity has been explained based on analyses of recombinant human mutant proteins or, more recently, in the context of structural models for the enzyme. We report the experimental...
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