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Am J Clin Nutr. 1993 Nov;58(5 Suppl):788S-795S. doi: 10.1093/ajcn/58.5.788S.

Inborn errors of fructose metabolism.

Author information

1
Department of Biochemistry and Molecular Biology, Medical College of Georgia, Augusta.

Abstract

A review is presented of genetic defects affecting fructose metabolism in humans. Presently, six conditions have been recognized: fructose malabsorption, fructokinase deficiency, aldolase A and aldolase B deficiency, fructose-1,6-diphosphatase deficiency and D-glyceric aciduria. Clinical presentations of these conditions, enzymatic and/or molecular defects, pathophysiological consequences, and modes of treatments are discussed.

PMID:
8213611
DOI:
10.1093/ajcn/58.5.788S
[Indexed for MEDLINE]

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