Navigation Links
A mouse model brings new perspectives on Lafora disease
Date:12/12/2011

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 them to degenerate. This neuronal deterioration and death associated with glycogen accumulation is the hallmark of an extremely rare and progressive type of epilepsy known as Lafora disease (LD).

The journal EMBO Molecular Medicine has just published online the new insights into LD provided by a team of Spanish researchers headed by Joan J. Guinovart, director of the IRB Barcelona and Professor at the University of Barcelona (UB), in collaboration with Agns Gruart and Jos M. Delgado at the Division of Neurosciences at the Pablo Olavide University in Seville, and Eduardo Soriano, Professor at the UB and researcher at IRB Barcelona.

LD is a fatal, inherited and progressive form of epilepsy that causes irreversible neurodegeneration and for which no cure or effective treatment is available. Onset typically occurs during late childhood or adolescence, generally starting with epileptic seizures, progressing into deterioration of neurological functions. One of the hallmarks of the disease is the abnormal accumulation of glycogen aggregates, known as Lafora bodies (LB), in neurons. In these cells, two genes, malin and laforin, act in unison to block glycogen storage in normal conditions. Mutations changes in DNA in either laforin or malin genes are responsible for this disease.

The group of researchers led by Guinovart has developed a genetically engineered mice line lacking the malin gene, thus mimicking human LD in this animal model. The results obtained confirm the role of the malin-laforin complex in the regulation of glycogen synthesis in neurons. Jordi Duran, first author of the study together with Jordi Valls, explains, "Brain cells control their glycogen levels carefully by employing a 'team of wardens
'/>"/>

Contact: Nuria Noriega
34-934-037-255
Institute for Research in Biomedicine (IRB Barcelona)
Source:Eurekalert

Page: 1 2

Related biology technology :

1. ExonHit Launches Mouse Genome SpliceArray(TM) for use in Human Disease Modeling Studies
2. Mouse study reveals genetic component of empathy
3. febit First to Offer Newly Published Mouse Genome on a Chip for DNA/RNA Detection and Enrichment
4. EnVivo Pharmaceuticals Announces Reversal of Cognitive Deficits in Alzheimers Disease Transgenic Mouse Model With Gamma-Secretase Modulator
5. ALPCO Introduces a Mouse HMW and Total Adiponectin ELISA
6. MD Bioproducts Releases a New Mouse T1/ST2 ELISA
7. Crescendo Biologics Announces First Triple Knockout Mouse
8. A knockout resource for mouse genetics
9. Researchers produce detailed map of gene activity in mouse brain
10. Campbell Alliance Sales Practice to Present at CBIs Leadership Summit on Evolving the Pharma Sales Model
11. Repligen Announces Publication of Positive Results with Proprietary HDAC Inhibitor in Huntingtons Disease Model
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:3/4/2015)... -- Brooklyn residents now have a new place to go ... Nunzio Saulle , M.D., a board-certified physiatrist, is ... Physical Medicine and Rehabilitation (PM&R) practice in ... Health Plus Management, LLC is managing the day-to-day operations ... Avenue. This new PM&R practice will treat patients suffering ...
(Date:3/4/2015)... , Mar. 04, 2015 Research and Markets ... addition of Jain PharmaBiotech,s new report "Biomarkers ... offering. This report follows the ... that can be objectively measured and evaluated as ... as well as pharmacological responses to a therapeutic ...
(Date:3/4/2015)... 2015 /CNW/ - Resverlogix Corp. (TSX:RVX) (the "Company") ... collaborative research program with Emerald Logic, a ... Evolution Technology (FACET), Emerald Logic analyzed Resverlogix,s ... from each of 798 patients who participated ... ASSERT, SUSTAIN and ASSURE. The objective of ...
(Date:3/4/2015)... Proove Biosciences , a ... excited to announce the launch of their CME-accredited ... Management of Pain”. , The continuing medical education ... next year. The focus of the program is ... pain treatments, integrate appropriate pain management techniques, and ...
Breaking Biology Technology:NJS Physical Medicine & Rehabilitation Opening on Flatbush Avenue, Brooklyn, NY 2Global Biomarkers Report 2014-2024 - Technologies, Markets and Companies 2Global Biomarkers Report 2014-2024 - Technologies, Markets and Companies 3Resverlogix Collaborates with Emerald Logic to Identify Factors Driving Drug Response and Efficacy in Phase 2 Program 2Resverlogix Collaborates with Emerald Logic to Identify Factors Driving Drug Response and Efficacy in Phase 2 Program 3Resverlogix Collaborates with Emerald Logic to Identify Factors Driving Drug Response and Efficacy in Phase 2 Program 4Proove Biosciences Launches CME Accredited Program with Medscape 2Proove Biosciences Launches CME Accredited Program with Medscape 3
... BERKELEY, Calif., March 28 Aerovance Inc. today,announced ... financing. Oxford,Finance Corporation led the syndicate, which includes ... $10 million from the financing line,at closing. The ... of,corporate milestones., "This financing reflects continued interest ...
... Joins Investor Syndicate, VIENNA, Austria, March ... novel antibodies and antibody fragments,based on its ... it,has acquired future royalty obligations to New ... Modular Antibody Technology was originally developed by ...
... HAMBURG, Germany and OXFORD, England, March 28 /PRNewswire-FirstCall/,-- ... financial,results according to IFRS for the year ended ... year were driven by strong progress in the,Company,s ... of Neuro3d, the sale of Evotec Technologies (ET), ...
Cached Biology Technology:Antibody Engineering Company F-Star Buys Back Royalty Obligations 2Evotec Announces Financial Results for 2007 2Evotec Announces Financial Results for 2007 3Evotec Announces Financial Results for 2007 4Evotec Announces Financial Results for 2007 5Evotec Announces Financial Results for 2007 6Evotec Announces Financial Results for 2007 7Evotec Announces Financial Results for 2007 8
(Date:1/22/2015)... Research and Markets ( http://www.researchandmarkets.com/research/4trb7t/biometrics_a ) has announced ... Overview" report to their offering. , ... an individual is who she/he is claiming to be, and ... person,s unique physical characteristics, such as fingerprint, hand or palm ...
(Date:1/22/2015)... Jan. 13, 2015 /PRNewswire/ - Today, FindBiometrics, the leading source of ... of its new website design. "When we launched ... says Peter O,Neill , founder and CEO of FindBiometrics. ... needs involvement from the key players on a very broad ...
(Date:1/22/2015)... Hall E -   EyeLock Inc. , a market leader ... its EyeLock ID technology integrated in a 3D printed automobile ... Ridge National Laboratory (ORNL) at the 2015 North ... is being used to validate the driver and authorize the ...
Breaking Biology News(10 mins):Biometrics - A Global Market Overview 2Biometrics - A Global Market Overview 3Identity Industry Leading Publication Debuts New Look As Biometrics Hit Mainstream 2EyeLock Debuts First Iris Authentication Technology Embedded in 3D Printed Automobile 2EyeLock Debuts First Iris Authentication Technology Embedded in 3D Printed Automobile 3
... In the first field trial of plants genetically tweaked ... plants handily beat out their wild-type counterparts. The results ... alternative for cleaning up polluted soil. , The new ... Science and Technology, show that three transgenic lines of ...
... Hotspots Revisited identifies 34 regions worldwide where 75 percent ... survive within habitat covering just 2.3 percent of the ... the five largest U.S. states). This habitat originally covered ... in size to Russia and Australia combined. The new ...
... novel bio-bar-code amplification (BCA) technology, researchers analyzing fluid from ... protein linked in recent studies to Alzheimer's disease. , ... : , - Gold nanoparticles and bio-bar codes ... Codes for the Ultrasensitive Detection of Proteins ,- ...
Cached Biology News:Transgenic plants remove more selenium from polluted soil than wild plants, new tests show 2Transgenic plants remove more selenium from polluted soil than wild plants, new tests show 3Transgenic plants remove more selenium from polluted soil than wild plants, new tests show 4Biodiversity hotspots identify conservation priorities 2Biodiversity hotspots identify conservation priorities 3Biodiversity hotspots identify conservation priorities 4Biodiversity hotspots identify conservation priorities 5Nanoscale Diagnostic Sets Sights on Alzheimer's 2Nanoscale Diagnostic Sets Sights on Alzheimer's 3
... is a high-quality four-place microplate shaker with ... speed is adjustable from 100 to 1350 ... a small orbit ensure effective shaking for ... quick and easy to set for 0 ...
... Ascent FL is equipped ... luminometric measurement technologies that ... of advanced features of ... Ascent. Fluoroskan Ascent FL ...
Rabbit polyclonal to STUB1 ( Abpromise for all tested applications). entrezGeneID: 10273 SwissProtID: Q9UNE7...
... stainless steel rack holders ... mechanism that permits selection ... angles, and "snap-in" product ... rack holder will accomodate ...
Biology Products: