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. 1977 May;90(5):746-50.
doi: 10.1016/s0022-3476(77)81240-7.

Glutaric aciduria: biochemical and morphologic considerations

S I Goodman, M D Norenberg, R H Shikes, D J Breslich, P G Moe

Glutaric aciduria: biochemical and morphologic considerations

S I Goodman et al. J Pediatr. 1977 May.

Abstract

Biochemical and morphologic studies on a patient with glutaric aciduria are presented. Generalized aminoaciduria, alpha-aminoadipic aciduria, and saccharopinuria were noted just prior to death, as well as glutaconic aciduria greater than beta-hydroxyglutaric aciduria. Mutant liver mitochondria did not oxidize glutaryl-CoA to glutaconyl-CoA, indicating deficiency of glytaryl-CoA dehydrogenase. Autopsy revealed cerebral edema, ischemic neuronal changes, and striatal degeneration in the brain with fatty changes in liver, kidney, and myocardium.

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