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American Journal of Clinical Nutrition, Vol 58, 788S-795S, Copyright © 1993 by The American Society for Clinical Nutrition, Inc
REVIEW ARTICLES |
FA Hommes
Department of Biochemistry and Molecular Biology, Medical College of Georgia, Augusta.
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.
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