Literature DB >> 7671974

Hidden sources of galactose in the environment.

P B Acosta1, K C Gross.   

Abstract

A galactose-restricted diet free of lactose is lifesaving in patients with galactose-1-phosphate uridyl transferase (GALT) deficiency, but does not prevent long-term complications such as developmental delay, abnormal speech, poor growth and, in females, ovarian failure. Lactose, found in dairy products and as an extender in drugs, has been considered the primary source of galactose in the diet. Two recent publications reported that small amounts of galactose are present in many fruits and vegetables. We report the presence of considerable amounts of free galactose in some legumes (dried beans and peas) and the presence of bound galactose in many food plants. Galactose, in various glycosidic linkages, such as alpha-1,6, beta-1,3 and beta-1,4, and as a component of lipids, is ubiquitous in animals and plants. The bioavailability of alpha-1,6 and beta-1,3 linked galactose in foods is unknown. However, alpha-galactosidases found in plant and animal tissues may release galactose in alpha-1,6 linkage, and from diagalactosyldiacylglycerol. Galactose in beta-1,4 linkage and as monogalactosyldiacylglycerol may be released by beta-galactosidases in animal and plant tissues. Foods fermented by microorganisms for preparation or preservation purposes may contain free galactose. The role of free and bound galactose in cereals, fruits, legumes, nuts, organ meats, seeds, and vegetables in the poor outcome seen in some patients with GALT deficiency is unknown. It is certain that no patients with GALT deficiency have ever ingested a galactose-free diet.

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Year:  1995        PMID: 7671974     DOI: 10.1007/bf02143811

Source DB:  PubMed          Journal:  Eur J Pediatr        ISSN: 0340-6199            Impact factor:   3.183


  19 in total

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  28 in total

1.  Recommendations for the management of galactosaemia. UK Galactosaemia Steering Group.

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Journal:  Arch Dis Child       Date:  1999-01       Impact factor: 3.791

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3.  Multiple GAL pathway gene clusters evolved independently and by different mechanisms in fungi.

Authors:  Jason C Slot; Antonis Rokas
Journal:  Proc Natl Acad Sci U S A       Date:  2010-05-17       Impact factor: 11.205

Review 4.  Innovative therapy for Classic Galactosemia - tale of two HTS.

Authors:  M Tang; S I Odejinmi; H Vankayalapati; K J Wierenga; K Lai
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Review 5.  Classical galactosaemia revisited.

Authors:  Annet M Bosch
Journal:  J Inherit Metab Dis       Date:  2006-07-11       Impact factor: 4.982

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Authors:  Allison B Frederick; David J Cutler; Judith L Fridovich-Keil
Journal:  J Inherit Metab Dis       Date:  2017-07-10       Impact factor: 4.982

7.  Selenite and ebselen supplementation attenuates D-galactose-induced oxidative stress and increases expression of SELR and SEP15 in rat lens.

Authors:  Jie Dai; Jun Zhou; Hongmei Liu; Kaixun Huang
Journal:  J Biol Inorg Chem       Date:  2016-10-17       Impact factor: 3.358

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Authors:  Rajaneesh Karimpurath Gopinath; Jun-Yi Leu
Journal:  Mol Cell Biol       Date:  2016-04-15       Impact factor: 4.272

9.  Gastrointestinal Health in Classic Galactosemia.

Authors:  Kelly A Shaw; Jennifer G Mulle; Michael P Epstein; Judith L Fridovich-Keil
Journal:  JIMD Rep       Date:  2016-07-01

10.  High-throughput screening for human galactokinase inhibitors.

Authors:  Klaas J Wierenga; Kent Lai; Peter Buchwald; Manshu Tang
Journal:  J Biomol Screen       Date:  2008-05-19
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