Featured Research

from universities, journals, and other organizations

Accumulation Of Sugar In Neurons May Explain Origin Of Several Neurodegenerative Diseases

Date:
October 22, 2007
Source:
Institute for Research in Biomedicine
Summary:
A phenomenon considered healthy for cells, such as the accumulation of long chains of glucose (glycogen), which tissues store for energy purposes, is harmful for neurons. The researchers made the discovery while studying Lafora disease, a rare pathology that causes irreversible neurodegeneration in adolescents and for which no treatment is available. Understanding the mechanisms that trigger and block the production of glycogen may be of great use to address the study of other neurodegenerative and neurological diseases.

Image obtained by confocal microscopy. The accumulation of glycogen (yellow and red) in neurons causes their degradation and cell suicide ensues.
Credit: Copyright IRB Barcelona

A phenomenon considered healthy for cells, such as the accumulation of long chains of glucose (glycogen), which tissues store for energy purposes, is harmful for neurons. This finding has been made by a team of Spanish researchers led by Joan J. Guinovart, director of the Institute for Research in Biomedicine (IRB Barcelona) and senior professor at the University of Barcelona (UB), and Santiago Rodríguez de Córdoba, research professor at the Centro Superior de Investigaciones Científicas (CSIC).

Related Articles


The researchers made the discovery while studying Lafora disease, a rare pathology that causes irreversible neurodegeneration in adolescents and for which no treatment is available. Lafora disease generally presents as epileptic seizures between 10 to 17 years of age and later on as myoclonus (involuntary twitching of the arms and legs). Its evolution is marked by progressive degeneration of the nervous system which reduces the patient to a terminal vegetative state ten years after its onset.

This disease is inherited from parents who are carriers of mutations in one of the two genes associated with the pathology. These genes are called laforin (named after Dr. Lafora) and malin (from the French expression "le grand mal", used to refer to epilepsy). The disease is characterized by the accumulation of abnormal inclusions, known as Lafora bodies, in neurons.

The study describes the function of laforin and malin, explains the origin of Lafora bodies and identifies how the neurodegenerative process of this disease arises. Joan J. Guinovart, expert in glycogen metabolism explains, "We have observed that laforin and malin act jointly as "guardians" of glycogen levels in neurons and are stimulated by the degradation of the proteins responsible for glucose accumulation. In a situation in which either of the two genes loses its function, these proteins are not degraded, glycogen accumulates and thus neurons deteriorate and cell suicide (apoptosis) ensues.

The conclusions of the study have increased expectations of finding a strategy to treat Lafora disease. One strategy consists of identifying a molecule with the capacity to inhibit glycogen synthesis in neurons.

The breakthroughs on the mechanisms that trigger and block the production of glycogen may be of great use to address the study of other neurodegenerative and neurological diseases. "We have extended the hypothesis of the study to other pathologies in which glycogen has been detected in neurons because our results suggest that this molecule is a part of the problem" comments Guinovart.

Spanish research contributions to Lafora disease have significantly improved our understanding of this pathology. These contributions date back to observations made by the physician Gonzalo Rodríguez Lafora, one of Santiago Ramón y Cajal's students, who, in 1911, discovered the presence of "Lafora bodies" in the nervous system of patients with the disease that carries his name. In 1999, the team headed by Rodríguez de Córdoba, together with José María Serratosa, identified the laforin gene.

Journal reference: Mechanism suppressing glycogen synthesis in neurons and its demise in progressive myoclonus epilepsy (2007) Vilchez D, Ros S, Cifuentes D, Pujadas Ll, Vallès J, García-Fojeda B, Criado-García O, Fernández-Sánchez E, Medraño-Fernández I, Domínguez J, García-Rocha M, Soriano E, Rodríguez de Córdoba S, GuinovartJ.J. Nature NeuroScience (doi 10.1038/nn1998)


Story Source:

The above story is based on materials provided by Institute for Research in Biomedicine. Note: Materials may be edited for content and length.


Cite This Page:

Institute for Research in Biomedicine. "Accumulation Of Sugar In Neurons May Explain Origin Of Several Neurodegenerative Diseases." ScienceDaily. ScienceDaily, 22 October 2007. <www.sciencedaily.com/releases/2007/10/071021142327.htm>.
Institute for Research in Biomedicine. (2007, October 22). Accumulation Of Sugar In Neurons May Explain Origin Of Several Neurodegenerative Diseases. ScienceDaily. Retrieved November 27, 2014 from www.sciencedaily.com/releases/2007/10/071021142327.htm
Institute for Research in Biomedicine. "Accumulation Of Sugar In Neurons May Explain Origin Of Several Neurodegenerative Diseases." ScienceDaily. www.sciencedaily.com/releases/2007/10/071021142327.htm (accessed November 27, 2014).

Share This


More From ScienceDaily



More Health & Medicine News

Thursday, November 27, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Ebola Leaves Orphans Alone in Sierra Leone

Ebola Leaves Orphans Alone in Sierra Leone

AFP (Nov. 27, 2014) — The Ebola epidemic sweeping Sierra Leone is having a profound effect on the country's children, many of whom have been left without any family members to support them. Duration: 01:02 Video provided by AFP
Powered by NewsLook.com
Experimental Ebola Vaccine Shows Promise In Human Trial

Experimental Ebola Vaccine Shows Promise In Human Trial

Newsy (Nov. 27, 2014) — A recent test of a prototype Ebola vaccine generated an immune response to the disease in subjects. Video provided by Newsy
Powered by NewsLook.com
Pet Dogs to Be Used in Anti-Ageing Trial

Pet Dogs to Be Used in Anti-Ageing Trial

Reuters - Innovations Video Online (Nov. 26, 2014) — Researchers in the United States are preparing to discover whether a drug commonly used in human organ transplants can extend the lifespan and health quality of pet dogs. Video provided by Reuters
Powered by NewsLook.com
Today's Prostheses Are More Capable Than Ever

Today's Prostheses Are More Capable Than Ever

Newsy (Nov. 26, 2014) — Advances in prosthetics are making replacement body parts stronger and more lifelike than they’ve ever been. Video provided by Newsy
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
 
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:  

Breaking News:

Strange & Offbeat Stories

 

Health & Medicine

Mind & Brain

Living & Well

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:  

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile iPhone Android Web
Follow Facebook Twitter Google+
Subscribe RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins