Advertisement

European Journal of Pediatrics

, Volume 154, Supplement 2, pp S87–S92 | Cite as

Hidden sources of galactose in the environment

  • Phyllis B. Acosta
  • Kenneth C. Gross
Article

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 α-1,6, β−1,3 and β−1,4, and as a component of lipids, is ubiquitous in animals and plants. The bioavailability of α−1,6 and β−1,3 linked galactose in foods is unknown. However, α-galactosidases found in plant and animal tissues may release galactose in α−1,6 linkage, and from digalactosyldiacylglycerol. Galactose in β−1,4 linkage and as monogalactosyldiacylglycerol may be released by β-galactosidases in animal and plant tissues. Foods fermented by micro-organisms 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.

Key words

Galactose Arabinogalactans Galactinol Galactopinitols Rhamnogalacturonans 

Abbreviations

Ara

arabinose

Gal-1-P

galactose-1-phosphate

Fru

fructose

Gal

galactose

GalA

galacturonic acid

Glc

glucose

GALT

galactose-1-phosphate uridyl transferase

Rha

rhamnose

References

  1. 1.
    Akinyele IO, Akinlosotu A (1991) Effect of soaking, dehulling and fermentation on oligosaccharides and nutrient content of cow peas (Vigna Unguiculata). Food Chem 41: 43–53CrossRefGoogle Scholar
  2. 2.
    Reference deletedGoogle Scholar
  3. 3.
    Aman P (1979) Carbohydrates in raw and germinated seeds from mung bean and chick pea. J Sci Food Agric 30: 869–875Google Scholar
  4. 4.
    Asp NG (1971) Human small-intestinal β-galactosidases. Separation and characterization of three forms of an acid β-galactosidase. Biochem J 191: 229–308Google Scholar
  5. 5.
    Aspinall GO (1980) Chemistry of cell wall polysaccharides. Biochem Plants 3: 473–499Google Scholar
  6. 6.
    Berry GT, Palmieri M, Gross KC, Acosta PB, Henstenburg JA, Mazur A, Reynolds R, Segal S (1993) The effect of dietary fruits and vegetables on urinary galactitol excretion in galactose-1-phosphate uridyltransferase deficiency. J Inherited Metab Dis 16: 91–100CrossRefPubMedGoogle Scholar
  7. 7.
    Budavari S (ed) (1989) Lactobionic acid. The Merck Index, 11th edn. Merck and Co, Inc. Rathway NJ, p 5224Google Scholar
  8. 8.
    Carter HE, McCluer RH, Slifer ED (1956) Lipids of wheat flour. I. Characterization of galactosylglycerol components. J Am Chem Soc 78: 3735–3738CrossRefGoogle Scholar
  9. 9.
    Carter HE, Hendry RA, Stanacev NZ (1961) Wheat flour lipids: Structure of the mono- and digalactosylglycerol lipids. J Lipid Res 2: 223–227Google Scholar
  10. 10.
    Carter HE, Ohno K, Nojima S, Tipton CL, Stanacev NZ (1961) Wheat flour lipids: II. Isolation and characterization of glycolipids of wheat flour and other plant sources. J Lipid Res 2: 215–222Google Scholar
  11. 11.
    Cegla GF, Bell KR (1977) High pressure liquid chromatography for the analysis of soluble carbohydrates in defatted oilseed flours. J Am Oil Chem Soc 54: 150–152Google Scholar
  12. 12.
    Cerbulis J (1954) Sugars in Caracas cocoa beans. Arch Biochem Biophys 49: 442–450CrossRefPubMedGoogle Scholar
  13. 13.
    Clarke AE, Anderson RL, Stone BA (1979) Form and function of arabinogalactans and arabinogalactan-proteins. Phytochemistry 18: 521–540CrossRefGoogle Scholar
  14. 14.
    Desnick RJ, Bishop DF (1989) Fabry disease: alpha-galactosidase deficiency; Schindler disease: alpha-N-acetylgalactoaminidase deficiency. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The metabolic basis of inherited disease, 6th edn. McGraw Hill, New York, pp 1751–1796Google Scholar
  15. 15.
    Dubois M, Geddes WF, Smith F (1960) The carbohydrates of the gramineae. X. A quantitative study of the carbohydrates of wheat gern. Cereal Chem 37: 557–568Google Scholar
  16. 16.
    Elbein AD (1980) Glycolipids. Biochem Plants 3: 571–587Google Scholar
  17. 17.
    Fincher GB, Stone BA, Clarke AE (1983) Arabinogalactan-proteins: Structure, biosynthesis, and function. Ann Rev Plant Physiol 34: 47–70CrossRefGoogle Scholar
  18. 18.
    Fry SC (1982) Phenolic components of the primary cell wall. Feruloylated disaccharides ofd-galactose andl-arabinose from spinach polysaccharide. Biochem J 203: 493–504PubMedGoogle Scholar
  19. 19.
    Fujino Y, Sakata S (1973) Glycerolglycolipids in rice grain. Cereal Chem 50: 379–382Google Scholar
  20. 20.
    Gitzelmann R, Auricchio S (1965) The handling of soy alpha-galactosides by a normal and a galactosemic child. Pediatrics 36: 231–234PubMedGoogle Scholar
  21. 21.
    Gitzelmann R, Hansen RG (1974) Galactose biogenesis and disposal in galactosemics. Biochim Biophys Acta 372: 374–378Google Scholar
  22. 22.
    Gross KC, Acosta PB (1991) Fruits and vegetables are a source of galactose: Implications in planning the diets of patients with galactosaemia. J Inherited Metab Dis 14: 253–258CrossRefPubMedGoogle Scholar
  23. 23.
    Gross KC, Watada AE, Kang MS, Kim SD, Kim KS, Lee SW (1986) Biochemical changes associated with the ripening of hot pepper fruit. Physiol Plant 66: 31–36Google Scholar
  24. 24.
    Gropper SS, Gross KC, Olds SJ (1993) Galactose content of selected fruit and vegetable baby foods: Implications for infants on galactose-restricted diets. J Am Diet Assoc 93: 328–330CrossRefPubMedGoogle Scholar
  25. 25.
    Harvey CD, Jenness R, Morris HA (1981) Gas chromatographic quantitation of sugars and nonvolatile watersoluble organic acids in commercial cheddar cheese. J Dairy Sci 64: 1648–1654Google Scholar
  26. 26.
    Hemavatahy J (1992) Lipid composition of melon (Cucumis melo) kernel. J Food Comp Anal 5: 90–95CrossRefGoogle Scholar
  27. 27.
    Hettinga DH, Miah AH, Hammond EG, Reinbold GW (1970) Sensitive enzymatic method for determination of glucose, galactose and lactose in cheddar cheese. J Dairy Sci 53: 1377–1380Google Scholar
  28. 28.
    Holton JB (1991) Galactosemia. In: Schaub J, Van Hoof F, Vis HL (eds) Inborn errors of metabolism. Raven Press, New York, pp 169–180Google Scholar
  29. 29.
    Jarvis MC (1984) Structure and properties of pectin gels in plant cell walls. Plant, Cell, Environ 7: 153–164Google Scholar
  30. 30.
    John MA, Dey PM (1986) Postharvest changes in fruit cell wall. Adv Food Res 30: 139–193Google Scholar
  31. 31.
    Knee M, Bartley JM (1981) Composition and metabolism of cell wall polysaccharides in ripening fruits. In: Friend J, Rhodes MJC (eds) Recent advances in the biochemistry of fruits and vegetables. Academic Press, New York, pp 133–148Google Scholar
  32. 32.
    Koch R, Acosta P, Ragsdale N, Donnell GN (1963) Nutrition in the treatment of galactosemia. J Am Diet Assoc 43: 216–222PubMedGoogle Scholar
  33. 33.
    Kumar A, Weatherly MR, Beaman DC (1991) Sweeteners, flavorings, and dyes in antibiotic preparations. Pediatrics 87: 352–360PubMedGoogle Scholar
  34. 34.
    Lepage M (1968) The lipid components of white potato tubers (Solanum tuberosum). Lipids 3: 477–481Google Scholar
  35. 35.
    Lineback DR, Ke CH (1975) Starches and low-molecular-weight carbohydrates from chick pea and horse bean flours. Cereal Chem 52: 334–347Google Scholar
  36. 36.
    Marlett JA (1992) Content and composition of dietary fiber in 117 frequently consumed foods. J Am Diet Assoc 92: 175–186PubMedGoogle Scholar
  37. 37.
    Matthews RH, Pehrsson PR, Farhat-Sabet M (1987) Sugar content of selected foods: individual and total sugars. Human Nutrition Information Service, USDA. U S Government Printing Office, Washington, DC, pp 1–39Google Scholar
  38. 38.
    McNeil M, Darvill AG, Fry SG, Albersheim P (1984) Structure and function of the primary cell walls of plants. Ann Rev Biochem 53: 625–663CrossRefPubMedGoogle Scholar
  39. 39.
    Minka S, Laurent G, Bruneteau M, Pivot V, Michel G (1991) Isolation and composition of glycolipids from corn flour. Food Chem 39: 329–336CrossRefGoogle Scholar
  40. 40.
    Naivikul O, D'Appolonia BL (1978) Comparison of legume and wheat flour carbohydrates. I. Sugar analysis. Cereal Chem 55: 913–918Google Scholar
  41. 41.
    Ng WG, Xu YK, Kaufam FR, Donnell GN (1989) Deficit of uridine diphosphate galactose in galactosaemia. J Inherited Metab Dis 12: 257–266CrossRefGoogle Scholar
  42. 42.
    Nguyen XV, Mazliak P (1990) Chilling injury induction is accompanied by galactolipid degradation in tomato pericarp. Plant Physiol Biochem 28: 283–291Google Scholar
  43. 43.
    O'Brien JS (1989) Beta-galactosidase deficiency (Gml gangliosidosis, galactosialidosis, and Morquio syndrome type B); ganglioside sialidase deficiency (mucolipidosis IV). In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The metabolic basis of inherited disease, 6th edn. McGraw Hill, New York, pp 1797–1839Google Scholar
  44. 44.
    Panoyotatos N, Villemez CL (1973) The formation of a beta-(1,4)-d-galactan chain catalyzed by aPhaseolus aureus enzyme. Biochem J 133: 263–271PubMedGoogle Scholar
  45. 45.
    Pressey R (1983) Beta-galactosidases in ripening tomatoes. Plant Physiol 71: 132–135Google Scholar
  46. 46.
    Pridham JB, Dey PM (1974) The nature and function of higher plant alpha-galactosidases. In: Pridham JB (ed) Plant carbohydrate biochemistry. Academic Press, New York, pp 83–96Google Scholar
  47. 47.
    Quemener B, Thibault JF (1990) Assessment of methanolysis for the determination of sugars in pectins. Carbohydrate Res 206: 277–287CrossRefGoogle Scholar
  48. 48.
    Reynolds LM, Henneberry GO, Baker BE (1959) Studies on Casein. II. The carbohydrate moiety of casein. J Dairy Sci 42: 1463–1471Google Scholar
  49. 49.
    Roelofsen PA (1958) Curing of cocoa beans. Adv Food Res 8: 225–296Google Scholar
  50. 50.
    Saunders RM (1970) The sugars of safflower. J Am Oil Chem Soc 47: 254–255Google Scholar
  51. 51.
    Schweizer TF, Horman I, Wursch P (1978) Low molecular weight carbohydrates from leguminous seeds: a new disaccharide: galactopinitol. J Sci Food Agric 29: 148–154Google Scholar
  52. 52.
    Segal S (1993) The challenge of galactosemia. Int Pediatr 8: 125–132Google Scholar
  53. 53.
    Selvendran RR (1984) The plant cell wall as a source of dietary fiber: chemistry and structure. Am J Clin Nutr 39: 320–337PubMedGoogle Scholar
  54. 54.
    Selvendran RR (1985) Developments in the chemistry and biochemistry of pectic and hemicellulosic polymers. J Cell Sci [Suppl] 2: 51–88Google Scholar
  55. 55.
    Smith JS, Villalobos MC, Kottemann CM (1986) Quantitative determination of sugars in various food products. J Food Sci 51: 1373–1375Google Scholar
  56. 56.
    Sosulski FW, Elkowicz L, Reichert RD (1982) Oligosaccharides in eleven legumes and their air-classified protein and starch fractions. J Food Sci 47: 498–502Google Scholar
  57. 57.
    Steffen CHR: (1975) Enzymatische Bestimmungsmethoden zur Erfassung der Gärungsvorgänge in der milchwirtschaftlichen Technologie. Lebensm Wiss U Technol 8: 1–6Google Scholar
  58. 58.
    Steffen CHR, Nick B, Blanc B (1975) Methodik zur enzymatischen Bestimmung von Laktose, Glukose, Galaktose und Laktat im Käse. Schweiz Milchw Forsch 4: 13–15Google Scholar
  59. 59.
    Steffen CHR, Nick B, Blanc C (1975) Die gärungstechnische Rolle von Laktose, Glukose und Galaktose in der Fabrikation von Emmentalerkäse. Schweiz Milchw Forsch 4: 16–22Google Scholar
  60. 60.
    Suzuki K, Suzuki Y (1989) Galactosylceramide lipidosis: globoid-cell leukodystrophy (Krabbe disease). In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The metabolic basis of inherited disease, 6th edn. McGraw Hill, New York, pp 1699–1720Google Scholar
  61. 61.
    Wood PJ, Siddiqui IR (1972) Isolation and structural studies of a water soluble galactan from potato (Solanum tuberosum) tubers. Carbohydrate Res 22: 212–220CrossRefGoogle Scholar
  62. 62.
    Waggoner DD, Buist NRM, Donnell GN (1990) Long-term prognosis in galactosaemia: Results of a survey of 350 cases. J Inherited Metab Dis 13: 802–818CrossRefPubMedGoogle Scholar
  63. 63.
    Yokotsuka T (1986) Soy sauce biochemistry. Adv Food Res 30: 195–329PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1995

Authors and Affiliations

  • Phyllis B. Acosta
    • 1
  • Kenneth C. Gross
    • 2
  1. 1.Ross Products DivisionAbbott LaboratoriesColumbusUSA
  2. 2.Horticultural Crops Quality Laboratory, Beltsville Agricultural Research Center, Agricultural Research ServiceUSDABeltsvilleUSA

Personalised recommendations