Glycogen accumulation in neurons causes brain damage and shortens the lives of flies and mice

May 2, 2012
This image shows a cerebellum sample from a healthy mouse. Credit: Jordi Duran (IRB Barcelona)

Collaborative research by groups headed by scientists Joan J. Guinovart and Marco Milán at the Institute for Research in Biomedicine (IRB Barcelona) has revealed conclusive evidence about the harmful effects of the accumulation of glucose chains (glycogen) in fly and mouse neurons.

These two animal models will allow scientists to address the genes involved in this harmful process and to find pharmacological solutions that allow disintegration of the accumulations or limitation of production. Advances in this direction would make a significant contribution to investigation into Lafora progressive myoclonic epilepsy and other neurodegenerative diseases characterized by glycogen accumulation in . The journal EMBO Molecular Medicine publishes the results of the study this week.

This image shows the same tissue (mouse cerebellum) after glycogen accumulation. Credit: Jordi Duran (IRB Barcelona)

"Our data clearly indicate that glycogen accumulation alone kills neurons and thus dramatically reduces lifespan", explains Guinovart, an expert in glycogen metabolism, group leader at IRB Barcelona, and senior professor at the University of Barcelona, "because the only thing we have manipulated in the neurons is their capacity to produce glycogen".

The inclusion of the Drosophila fly in the study provides in vivo confirmation of the theory in another animal model as these flies also show the same symptoms of degeneration as mice when glycogen accumulates in neurons. However, in addition the use of Drosophila will speed up obtaining genetic data and the screening of therapeutic molecules. "In a short time we will be able to perform a massive search for genes involved in the pathological process and to understand it better at the molecular level", emphasizes Marco Milán, ICREA researcher at IRB Barcelona and a specialist in Drosophila. "But the flies will also be useful to identify pharmacological molecules that can cure", he explains.

The IRB Barcelona teams are designing several experiments to identify the possible therapeutic targets that may be useful to prevent glycogen accumulation in neurons. In addition to the direct relation to Lafora epilepsy, a progressive degenerative disease that affects adolescents and has no cure, glycogen accumulation could be the main cause of other neurodegenerative illnesses such as Adult polyglucosan body disease and Andersen's disease.

Explore further: Mouse model brings new perspectives on Lafora disease

Related Stories

Mouse model brings new perspectives on Lafora disease

August 29, 2011

Short-term energy storage in animal cells is usually achieved through the accumulation of glucose, in the form of long and branched chains, known as glycogen. But when this accumulation happens in neurons it is fatal, causing ...

Recommended for you

MRI technique induces strong, enduring visual association

June 30, 2016

Researchers have made two new scientific points with a set of experiments in which they induced people to perceive colors that weren't really there—one concerning how the brain works and the other concerning how to work ...

Erasing unpleasant memories with a genetic switch

June 30, 2016

Researchers from KU Leuven (Belgium) and the Leibniz Institute for Neurobiology (Germany) have managed to erase unpleasant memories in mice using a 'genetic switch'. Their findings were published in Biological Psychiatry.

Motivation to bully is regulated by brain reward circuits

June 29, 2016

Individual differences in the motivation to engage in or to avoid aggressive social interaction (bullying) are mediated by the basal forebrain, lateral habenula circuit in the brain, according to a study conducted at the ...

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.