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Galactitol in the Tissues of a Galactosemic Child

Galactitol in the Tissues of a Galactosemic Child Abstract GALACTITOL has recently been isolated from the urine1 and brain2 of galactosemia patients. Subsequently the conversion of orally administered galactose- 1-C14 into galactitol-1-C14 by a 17-year-old male galactosemia patient was demonstrated.3 In order to account for the relatively slow excretion of administered radioactivity as galactitol-C14 it was suggested that there had either been deposition of galactitol in tissues of the body, or that the administered galactose-C14 had been incorporated into galactose-containing compounds. This hypothesis was further supported by the finding of galactitol in a variety of tissues in a rat fed large amounts of galactose.4 We now wish to report the galactitol, glucose, free, and phosphatide-bound myo-inositol content of body tissue from 17 areas of an infant presumed to be galactosemic. Material and Methods The patient studied was a 23-day-old male infant who was jaundiced on the third day of life. References 1. Wells, W.W.; Pittman, T.A.; and Egan, T.J.: The Isolation and Identification of Galactitol From the Urine of Patients With Galactosemia , J Biol Chem 239:3192, 1964. 2. Wells, W.W., et al: The Isolation and Identification of Galactitol From the Brains of Galactosemia Patients , J Biol Chem 240:1002, 1965. 3. Egan, T.J., and Wells, W.W.: Alternate Metabolic Pathway in Galactosemia: Observations in a Patient With Galactosemia , Amer J Dis Child 111: 400, 1966.Crossref 4. Quan-Ma, R., and Wells, W.W.: The Distribution of Galactitol in Tissues of Rats Fed Galactose , Biochem Biophys Res Commun 20:486, 1965.Crossref 5. Folch, J.; Lees, M.; and Sloane-Stanley, G.H.: A Simple Method for the Isolation and Purification of Total Lipides From Animal Tissues , J Biol Chem 226:497, 1957. 6. Wells, W.W.; Chin, T.; and Weber, B.: Quantitative Analysis of Serum and Urine Sugars by Gas Chromatography , Clin Chim Acta 10:352, 1964.Crossref 7. Wells, W.W.; Pittman, T.A.; and Wells, H.J.: Quantitative Analysis of Myoinositol in Rat Tissue by Gas-Liquid Chromatography , Anal Biochem 10: 450, 1965.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

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References (7)

Publisher
American Medical Association
Copyright
Copyright © 1966 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1966.02090140149018
Publisher site
See Article on Publisher Site

Abstract

Abstract GALACTITOL has recently been isolated from the urine1 and brain2 of galactosemia patients. Subsequently the conversion of orally administered galactose- 1-C14 into galactitol-1-C14 by a 17-year-old male galactosemia patient was demonstrated.3 In order to account for the relatively slow excretion of administered radioactivity as galactitol-C14 it was suggested that there had either been deposition of galactitol in tissues of the body, or that the administered galactose-C14 had been incorporated into galactose-containing compounds. This hypothesis was further supported by the finding of galactitol in a variety of tissues in a rat fed large amounts of galactose.4 We now wish to report the galactitol, glucose, free, and phosphatide-bound myo-inositol content of body tissue from 17 areas of an infant presumed to be galactosemic. Material and Methods The patient studied was a 23-day-old male infant who was jaundiced on the third day of life. References 1. Wells, W.W.; Pittman, T.A.; and Egan, T.J.: The Isolation and Identification of Galactitol From the Urine of Patients With Galactosemia , J Biol Chem 239:3192, 1964. 2. Wells, W.W., et al: The Isolation and Identification of Galactitol From the Brains of Galactosemia Patients , J Biol Chem 240:1002, 1965. 3. Egan, T.J., and Wells, W.W.: Alternate Metabolic Pathway in Galactosemia: Observations in a Patient With Galactosemia , Amer J Dis Child 111: 400, 1966.Crossref 4. Quan-Ma, R., and Wells, W.W.: The Distribution of Galactitol in Tissues of Rats Fed Galactose , Biochem Biophys Res Commun 20:486, 1965.Crossref 5. Folch, J.; Lees, M.; and Sloane-Stanley, G.H.: A Simple Method for the Isolation and Purification of Total Lipides From Animal Tissues , J Biol Chem 226:497, 1957. 6. Wells, W.W.; Chin, T.; and Weber, B.: Quantitative Analysis of Serum and Urine Sugars by Gas Chromatography , Clin Chim Acta 10:352, 1964.Crossref 7. Wells, W.W.; Pittman, T.A.; and Wells, H.J.: Quantitative Analysis of Myoinositol in Rat Tissue by Gas-Liquid Chromatography , Anal Biochem 10: 450, 1965.Crossref

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Nov 1, 1966

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