Are there errors in glycogen biosynthesis and is laforin a repair enzyme?

Research output: Contribution to journalArticle

15 Citations (Scopus)

Abstract

Glycogen, a branched polymer of glucose, is well known as a cellular reserve of metabolic energy and/or biosynthetic precursors. Besides glucose, however, glycogen contains small amounts of covalent phosphate, present as C2 and C3 phosphomonoesters. Current evidence suggests that the phosphate is introduced by the biosynthetic enzyme glycogen synthase as a rare alternative to its normal catalytic addition of glucose units. The phosphate can be removed by the laforin phosphatase, whose mutation causes a fatal myoclonus epilepsy called Lafora disease. The hypothesis is that glycogen phosphorylation can be considered a catalytic error and laforin a repair enzyme.

Original languageEnglish
Pages (from-to)3216-3218
Number of pages3
JournalFEBS Letters
Volume585
Issue number20
DOIs
StatePublished - Oct 20 2011

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Biosynthesis
Glycogen
Repair
Phosphates
Lafora Disease
Enzymes
Myoclonic Epilepsy
Glucose
Glycogen Synthase
Phosphorylation
Glucans
Phosphoric Monoester Hydrolases
Mutation

ASJC Scopus subject areas

  • Biochemistry
  • Biophysics
  • Cell Biology
  • Genetics
  • Molecular Biology
  • Structural Biology

Cite this

Are there errors in glycogen biosynthesis and is laforin a repair enzyme? / Roach, Peter.

In: FEBS Letters, Vol. 585, No. 20, 20.10.2011, p. 3216-3218.

Research output: Contribution to journalArticle

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