Journal
FEBS LETTERS
Volume 579, Issue 22, Pages 5079-5083Publisher
WILEY
DOI: 10.1016/j.febslet.2005.08.013
Keywords
UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosaminekinase; N-acetylmannosamine
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Sialic acids are expressed as terminal sugars in many glycoconjugates and play an important role during development and regeneration, as they are involved as polysialic acid in a variety of cell-cell interactions mediated by the neural cell adhesion molecule NCAM. The key enzyme for the biosynthesis of sialic acid is the UDP-N-acetylglucosamine 2-epimerase/N-acetyl-mannosamine-kinase (GNE). Mutations in the binding site of the feedback inhibitor CMP-sialic acid of the GNE leads to sialuria, a disease in which patients produce sialic acid in gram scale. Here, we report on the consequences after expression of a sialuria-mutated GNE. Expression of the sialuria-mutated GNE leads to a dramatic increase of both cellular sialic acid and polysialic acid on NCAM. This could also be achieved by application of the sialic acid precursor N-acetylmannosamine. Our data suggest that biosynthesis of sialic acid regulates and limits the synthesis of polysialic acid. (c) 2005 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.
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