HMG-CoA synthase deficiency

HMG-CoA synthase deficiency

Metabolic disease A condition caused by a congenital defect in mitochondrial HMG-CoA synthase, resulting in a poor tolerance to fasting, which may evoke hypoketosis–since HMG-CoA is pivotal in the control of ketogenesis Management Prevent fasting. See HMG-CoA synthase.