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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Trehalase deficiency


Other Names for this Disease
  • Trehalose intolerance
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Overview


Trehalase deficiency is a metabolic condition in which the body is not able to convert trehalose, a disaccharide found in mushrooms, algae and insects, into glucose.[1][2][3] Individuals with this deficiency experience vomiting, abdominal discomfort and diarrhea after eating foods containing trehalose.[4][5] Studies suggest that this condition is very rare in white Americans, very frequent in Greenland natives (where it has been reported in 8% of the population), and not infrequent among Finns.[2][3][4][6] However, since ingestion of large quantities of foods containing trehalose in not common, the real frequency in unknown. Most cases appear to be inherited in an autosomal recessive manner.[2] 
Last updated: 2/4/2010

References

  1. Murray IA, Coupland K, Smith JA, Ansell ID, Long RG. British Journal of Nutrition. 2000; http://journals.cambridge.org/action/displayFulltext?type=1&fid=880176&jid=BJN&volumeId=83&issueId=03&aid=880164. Accessed 2/4/2010.
  2. Semenza G, Auricchio S, Mantei N. Chapter 75 - Small Intestinal Disaccharidoses. The Metabolic & Molecular Bases of Inherited Disease, 8th ed. New York: McGraw-Hill; 2001;
  3. Abbott PJ, Chen J. WHO Food Additives Series 46: Trehalose. International Programme on Chemical Safety. http://www.inchem.org/documents/jecfa/jecmono/v46je05.htm. Accessed 2/4/2010.
  4. Kleinman RE, Goulet O, Mieli-Vergani G, Sherman PM. Walker's Pediatric Gastrointestinal Disease: Physiology, Diagnosis, Management, 5th edition. 2008;
  5. Trehalase Deficiency. Online Mendelian Inheritance in Man (OMIM). 2008; http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=612119. Accessed 2/4/2010.
  6. Swallow DM, Poulter M, Hollox EJ. Drug Metabolism and Disposition. 2001; http://dmd.aspetjournals.org/content/29/4/513.full. Accessed 2/4/2010.
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In Depth Information

  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Trehalase deficiency. Click on the link to view a sample search on this topic.