Deleterious effects of neuronal accumulation of glycogen in flies and mice

to related objects

Abstract: Under physiological conditions, most neurons keep glycogen synthase (GS) in an inactive form and do not show detectable levels of glycogen. Nevertheless, aberrant glycogen accumulation in neurons is a hallmark of patients suffering from Lafora disease or other polyglucosan disorders. Although these diseases are associated with mutations in genes involved in glycogen metabolism, the role of glycogen accumulation remains elusive. Here, we generated mouse and fly models expressing an active form of GS to force neuronal accumulation of glycogen. We present evidence that the progressive accumulation of glycogen in mouse and Drosophila neurons leads to neuronal loss, locomotion defects and reduced lifespan. Our results highlight glycogen accumulation in neurons as a direct cause of neurodegeneration.

Location
Deutsche Nationalbibliothek Frankfurt am Main
Extent
Online-Ressource
Language
Englisch

Bibliographic citation
Deleterious effects of neuronal accumulation of glycogen in flies and mice ; volume:4 ; number:8 ; year:2012 ; pages:719-729 ; extent:11
EMBO molecular medicine / European Molecular Biology Organization ; 4, Heft 8 (2012), 719-729 (gesamt 11)

Creator
Duran, Jordi
Tevy, María Florencia
Garcia‐Rocha, Mar
Calbó, Joaquim
Milán, Marco
Guinovart, Joan J.

DOI
10.1002/emmm.201200241
URN
urn:nbn:de:101:1-2023022807250491657812
Rights
Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
Last update
14.08.2025, 10:49 AM CEST

Data provider

This object is provided by:
Deutsche Nationalbibliothek. If you have any questions about the object, please contact the data provider.
Show original at data provider

Associated

  • Duran, Jordi
  • Tevy, María Florencia
  • Garcia‐Rocha, Mar
  • Calbó, Joaquim
  • Milán, Marco
  • Guinovart, Joan J.

Other Objects (12)

No signal of deleterious mutation accumulation in conserved gene sequences of extant asexual hexapods

No signal of deleterious mutation accumulation in conserved gene sequences of extant asexual hexapods

Proteinexpression adulter neuronaler Stammzellen bei Inhibition der Glycogen-Synthase-Kinase-3

Hochschulschrift

Proteinexpression adulter neuronaler Stammzellen bei Inhibition der Glycogen-Synthase-Kinase-3

Flies

Flies

Differential pattern of glycogen accumulation after protein phosphatase 1 glycogen-targeting subunit PPP1R6 overexpression, compared to PPP1R3C and PPP1R3A, in skeletal muscle cells

Differential pattern of glycogen accumulation after protein phosphatase 1 glycogen-targeting subunit PPP1R6 overexpression, compared to PPP1R3C and PPP1R3A, in skeletal muscle cells

Flies, Martin

Archivale

Flies, Martin

Flies, Josef

Personenakten

Flies, Josef

Flies, Josef

Personenakte

Flies, Josef

Hexokinase 2 drives glycogen accumulation in equine endometrium at day 12 of diestrus and pregnancy

Hexokinase 2 drives glycogen accumulation in equine endometrium at day 12 of diestrus and pregnancy

Increased biomass production and glycogen accumulation in apcE gene deleted Synechocystis sp. PCC 6803

Increased biomass production and glycogen accumulation in apcE gene deleted Synechocystis sp. PCC 6803

Hyper-hippocampal glycogen induced by glycogen loading with exhaustive exercise

Hyper-hippocampal glycogen induced by glycogen loading with exhaustive exercise

Influence of in situ progressive N-terminal is still controversial truncation of glycogen branching enzyme in Escherichia coli DH5α on glycogen structure, accumulation, and bacterial viability

Influence of in situ progressive N-terminal is still controversial truncation of glycogen branching enzyme in Escherichia coli DH5α on glycogen structure, accumulation, and bacterial viability

Der polnische Flies

Druck | Fotografie

Der polnische Flies

Related objects