World Library  
Flag as Inappropriate
Email this Article


Article Id: WHEBN0030290624
Reproduction Date:

Title: Galactolysis  
Author: World Heritage Encyclopedia
Language: English
Subject: Glycolysis, Fructolysis, Metabolism, P/O ratio, Anoxygenic photosynthesis
Collection: Glycolysis
Publisher: World Heritage Encyclopedia


Galactolysis refers to the catabolism of galactose.

In the liver, galactose is converted through the Leloir pathway to glucose 6-phosphate in the following reactions:

       galacto-                uridyl                phosphogluco-
        kinase               transferase                mutase
   gal --------> gal 1 P ------------------> glc 1 P -----------> glc 6 P
                            ^           \
                           /             v
                        UDP-glc       UDP-gal
                           ^             /

Metabolic disorders

There are 3 types of galactosemia or galactose deficiencies:

Name Enzyme Description
galactokinase deficiency Galactokinase Causes cataracts, which are treatable by restricting galactose from the diet.
UDPgalactose-4-epimerase deficiency UDPgalactose-4-epimerase Is extremely rare (only 2 reported cases). It causes nerve deafness.
Galactose-1-phosphate uridyl transferase deficiency Galactose-1-phosphate uridyl transferase Is the most problematic, as galactose-free diets are not effective in treating neurocognitive deficiencies (in particular language disorders such as verbal dyspraxia) and ovarian failure. If a galactose-free diet is administered, cataracts and acute symptoms such as kidney and liver failure respond immediately.

This article was sourced from Creative Commons Attribution-ShareAlike License; additional terms may apply. World Heritage Encyclopedia content is assembled from numerous content providers, Open Access Publishing, and in compliance with The Fair Access to Science and Technology Research Act (FASTR), Wikimedia Foundation, Inc., Public Library of Science, The Encyclopedia of Life, Open Book Publishers (OBP), PubMed, U.S. National Library of Medicine, National Center for Biotechnology Information, U.S. National Library of Medicine, National Institutes of Health (NIH), U.S. Department of Health & Human Services, and, which sources content from all federal, state, local, tribal, and territorial government publication portals (.gov, .mil, .edu). Funding for and content contributors is made possible from the U.S. Congress, E-Government Act of 2002.
Crowd sourced content that is contributed to World Heritage Encyclopedia is peer reviewed and edited by our editorial staff to ensure quality scholarly research articles.
By using this site, you agree to the Terms of Use and Privacy Policy. World Heritage Encyclopedia™ is a registered trademark of the World Public Library Association, a non-profit organization.

Copyright © World Library Foundation. All rights reserved. eBooks from Project Gutenberg are sponsored by the World Library Foundation,
a 501c(4) Member's Support Non-Profit Organization, and is NOT affiliated with any governmental agency or department.