Hydroxy butyric aciduria

Methyl 3-hydroxy butyric aciduria 2-Methyl 3-hydroxy butyryl-CoA dehydrogenase... 

Hydroxy butyric aciduria Succinic semialdehyde dehydrogenase... 

Greatly increased lactic aciduria is generally associated with an increased excretion of 2-hydroxy-n-butyric acid (Pettersen et al., 1973 Landaas and Pettersen, 1975) irrespective of the underlying cause of the increased lactate excretion. This acid is thought to arise from 2-oxobutyric acid, a metabolite of methionine, homocystine, cystathionine, serine and threonine, owing to the effect of the changes in NADH/NAD+ ratio in lactic acidosis (Pettersen etaL, 1973). The occurrence of 2-hydroxybutyrate in urine would thus imply an imbalance in cytosolic NADH/NAD and this metabolite may be of value in the further assessment of patients with lactic acidosis. 

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