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Polyglucosan body structure in Lafora disease.

M Kathryn Brewer1, Jean-Luc Putaux2, Alberto Rondon3

  • 1Department of Molecular and Cellular Biochemistry, University of Kentucky College of Medicine, Lexington, KY, 40536, USA; Lafora Epilepsy Cure Initiative, Epilepsy and Brain Metabolism Center, and Center for Structural Biology, University of Kentucky College of Medicine, Lexington, KY, 40536, USA; Institute for Research in Biomedicine (IRB Barcelona), 08028, Barcelona, Spain.

Carbohydrate Polymers
|June 2, 2020
PubMed
Summary

Polyglucosan bodies (PGBs), abnormal carbohydrate structures, are linked to neurological disorders. This study defines the architecture and formation of Lafora bodies (LBs), a type of PGB in Lafora disease, offering insights into PGBs in aging and neurological conditions.

Keywords:
Lafora bodiesLafora diseaseepilepsyglycogenglycogen storage diseasepolyglucosan bodiesstarch

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Area of Science:

  • Biochemistry
  • Cell Biology
  • Neuroscience

Background:

  • Abnormal carbohydrate structures called polyglucosan bodies (PGBs) are implicated in neurological disorders, glycogen storage diseases (GSDs), and aging.
  • Lafora bodies (LBs), a type of PGB, are characteristic of Lafora disease (LD), a fatal childhood epilepsy linked to EPM2A or EPM2B gene mutations.
  • LBs arise from disrupted glycogen metabolism and are found in the brain, muscle, and heart of LD patients and affected mouse models.

Purpose of the Study:

  • To elucidate the architectural features and tissue-specific variations of Lafora bodies (LBs).
  • To investigate the dynamic processes involved in LB formation.
  • To propose a model for the aggregation of small polyglucosans into mature LBs.

Main Methods:

  • Histological analysis of LBs in affected tissues.
  • Comparative studies of PGBs across different conditions.
  • Development of a theoretical model for LB aggregation.

Main Results:

  • Detailed characterization of LB architecture and identification of tissue-specific differences.
  • Insights into the dynamic assembly of LBs from smaller polyglucosan precursors.
  • A proposed model for polyglucosan body formation.

Conclusions:

  • Lafora bodies share structural similarities with PGBs found in aging and other neurological disorders.
  • Understanding LB formation provides generalizable insights into PGB structure and aggregation mechanisms.
  • This research contributes to the broader understanding of carbohydrate metabolism disorders and neurodegeneration.