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Description

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3-Hydroxy-3-methylglutaryl-CoA lyase deficiency is a disorder biochemically characterized by the predominant accumulation of 3-hydroxy-3-methylglutarate (HMG), 3-methylglutarate (MGA), 3-methylglutaconate and 3-hydroxyisovalerate in tissues and in vivo administration of HMG and MGA significantly decrease GSH concentration in liver in young rats [25]