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Review
. 2015 Feb 4:6:14.
doi: 10.3389/fneur.2015.00014. eCollection 2015.

Changing shapes of glycogen-autophagy nexus in neurons: perspective from a rare epilepsy

Pankaj Kumar Singh  1 , Sweta Singh  1
Affiliations
Review

Changing shapes of glycogen-autophagy nexus in neurons: perspective from a rare epilepsy

Pankaj Kumar Singh et al. Front Neurol. .

Abstract

In brain, glycogen metabolism is predominantly restricted to astrocytes but it also indirectly supports neuronal functions. Increased accumulation of glycogen in neurons is mysteriously pathogenic triggering neurodegeneration as seen in "Lafora disease" (LD) and in other transgenic animal models of neuronal glycogen accumulation. LD is a fatal neurodegenerative disorder with excessive glycogen inclusions in neurons. Autophagy, a pathway for bulk degradation of obsolete cellular constituents also degrades metabolites like lipid and glycogen. Recently, defects in this pathway emerged as a plausible reason for glycogen accumulation in neurons in LD, although some contradictions prevail. Albeit surprising, a reciprocal regulation of autophagy by glycogen in neurons has also just been proposed. Notably, increasing evidences of interaction between proteins of autophagy and glycogen metabolism from diverse model systems indicate a conserved, dynamic, and regulatory cross-talk between these two pathways. Concerning these findings, we herein provide certain models for the molecular basis of this cross-talk and discuss its potential implication in the pathophysiology of LD.

Keywords: Lafora disease; autophagy; glycogen; neurodegeneration; polyglucosan.

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Figures

Figure 1
Figure 1
A model of autophagy–glycogen proteins interaction based on current understanding and its potential implication in Lafora disease (LD). The schematic representation shows the identified interaction until now between the proteins of glycogen metabolism and autophagy network. The physiological implication of these interactions in context of Lafora disease (LD) is discussed in this review.
See this image and copyright information in PMC

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