Structural Mechanism of Laforin Function in Glycogen Dephosphorylation and Lafora Disease

No abstract is available for this paper.

• Publication year

  2015

• Venue

  Molecules and Cells

• Publication date

  2015-01-22

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1016/j.molcel.2014.11.020PMID 25544560PMCID PMC4337892
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Does abnormal glycogen structure contribute to increased susceptibility to seizures in epilepsy?

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• Does abnormal glycogen structure contribute to increased susceptibility to seizures in epilepsy?

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  2014influential reference
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• Starch Metabolism in Arabidopsis

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• Glycogen and its metabolism: some new developments and old themes.

  2012influential reference
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• Abnormal Metabolism of Glycogen Phosphate as a Cause for Lafora Disease*

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• Mechanism suppressing glycogen synthesis in neurons and its demise in progressive myoclonus epilepsy

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  1998cited by this paper
• Microscopy of starch : evidence of a new level of granule organization

  1997cited by this paper
• Physiologic coupling of glial glycogen metabolism to neuronal activity in brain.

  1992cited by this paper
• Progressive myoclonus epilepsies: clinical and neurophysiological diagnosis.

  1991cited by this paper
• Studies in myoclonus epilepsy (Lafora body form). I. Isolation and preliminary characterization of Lafora bodies in two cases.

  1968cited by this paper

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