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:4/28/2016)... BANGALORE, India , April 28, 2016 ... of Infosys (NYSE: INFY ), and Samsung SDS, ... partnership that will provide end customers with a more ... payment services.      (Logo: http://photos.prnewswire.com/prnh/20130122/589162 ) ... financial services, but it also plays a fundamental part in ...
(Date:4/28/2016)... First quarter 2016:   , Revenues amounted ... quarter of 2015 The gross margin was 49% (27) ... the operating margin was 40% (-13) Earnings per share ... operations was SEK 249.9 M (21.2) , Outlook   ... M. The operating margin for 2016 is estimated to ...
(Date:4/26/2016)... and LONDON , April ... part of EdgeVerve Systems, a product subsidiary of ... today announced a partnership to integrate the Onegini ...      (Logo: http://photos.prnewswire.com/prnh/20151104/283829LOGO ) ... their customers enhanced security to access and transact ...
Breaking Biology News(10 mins):
(Date:4/27/2016)... 2016 NanoStruck Technologies Inc. ... ( Frankfurt : 8NSK) gibt bekannt, ... 13. August 2015 die Genehmigung von der CNSX ... 200.000.000 Einheiten auf 400.000.000 Einheiten zu erhöhen, um ... wurden 157.900.000 Einheiten mit dem ersten Teil der ...
(Date:4/27/2016)... ... April 27, 2016 , ... NDA Partners ... the company as an Expert Consultant. Mr. Clark was formerly a Vice ... the development of small molecule monographs based on analytical methods. NDA Partners ...
(Date:4/27/2016)... ... April 27, 2016 , ... Global Stem Cells Group ... Ross is the founder of GSCG affiliate Kimera Labs in Miami. , In 2004, ... hematopoietic stem cell transplantation for hematologic disorders and the suppression of graft vs. host ...
(Date:4/27/2016)... ReportsnReports.com adds 2016 global ... on US, EU, China ... healthcare business intelligence collection of its growing online ... on the Flow Cytometry market spread across 153 ... tables and figures is now available at ...
Breaking Biology Technology: