Navigation Links
The accumulation of sugar in neurons may explain the origin of several neurodegenerative diseases
Date:10/21/2007

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. Published in the latest issue of Nature Neuroscience, 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 Rodrguez de Crdoba, research professor at the Centro Superior de Investigaciones Cientficas (CSIC). This research has been possible thanks to close collaboration between these two groups, who, in addition, have been assisted by neurobiology expert Eduardo Soriano, who is also a researcher at IRB Barcelona and senior professor at the UB.

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 Rodrguez Lafora, one of Santiago Ramn y Cajals 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 Rodrguez de Crdoba, together with Jos Mara Serratosa, identified the laforin gene.


'/>"/>

Contact: Sonia Armengou
sonia.armengou@irbbarcelona.org
34-934-037-255
Institute for Research in Biomedicine (IRB Barcelona)
Source:Eurekalert

Related biology news :

1. Thai spice helps cut blood sugar swings
2. Sugar beet virus mutation requires Texas touch
3. Cats indifference towards sugar explained
4. Sugar helps control cell division
5. Life-extending protein keeps blood sugar in check
6. Too much sugar not good for coral reefs
7. Darkness unveils vital metabolic fuel switch between sugar and fat
8. Scientists seek to unwrap the sweet mystery of the sugar coat on bacteria
9. A single sugar found responsible for an antibodys ability to treat inflammation
10. Researchers attack tumor cells by exploiting dependency on sugar metabolism
11. Acid rain causing decline in sugar maples, say researchers
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:2/2/2016)... , Feb. 2, 2016 This ... the bioinformatic market by reviewing the recent advances ... tools that drive the field forward. Includes forecast ... Identify the challenges and opportunities that exist ... and software solution developers, as well as IT ...
(Date:2/1/2016)... 2016 Rising sales of consumer ... touchfree intuitive gesture control market size ... of consumer electronics coupled with new technological advancements to ... size through 2020   --> ... technological advancements to drive global touchfree intuitive gesture control ...
(Date:1/25/2016)... , Jan. 25, 2016   Unisys Corporation (NYSE: ... John F. Kennedy (JFK) International Airport, New York City ... (CBP) identify imposters attempting to enter the United States ... to them. pilot testing of the system at ... three terminals at JFK during January 2016. --> pilot ...
Breaking Biology News(10 mins):
(Date:2/10/2016)... - BioAmber Inc. (NYSE: BIOA ), a leader ... & Co. Ltd., its partner in the ... an additional CDN$25 million in the joint venture for ... to 40%.  Mitsui will also play a stronger role ... Sarnia , providing dedicated resources alongside BioAmber,s ...
(Date:2/10/2016)... ASAE is introducing a hybrid membership model ... the option of joining or renewing through an organizational ... staff size, every employee in any size association or ... all available member benefits.   John H. ... options will allow organizations of any size and their ...
(Date:2/10/2016)... -- Matchbook, Inc., a company specializing in procurement and ... today the appointment of Jim Shuman ... 25 years of experience in supply chain, logistics ... decades in executive level roles as the SVP ... most recently headed global logistics and procurement at ...
(Date:2/10/2016)... ... February 10, 2016 , ... SonaCare Medical, LLC ... support program, Sonalinkā„¢ remote monitoring. The inaugural launch of this new technology occurred ... connecting Dr. Samuel Peretsman to a HIFU technical expert at SonaCare Medical headquarters. ...
Breaking Biology Technology: