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:5/1/2015)... ALBANY, N.Y. , May 1, 2015 ... today that William S. Marth , AMRI,s President ... of America Merrill Lynch 2015 Health Care Conference on ... A live audio webcast of the presentation can ... http://ir.amriglobal.com/events.cfm?CalendarID=2          The webcast will be ...
(Date:4/30/2015)... WARREN, N.J. , April 30, 2015  Roka ... company focused on providing advanced testing solutions for the ... financial results for the first quarter of 2015 on ... markets. Roka Bioscience will also host a ... 7, 2015 to discuss its first quarter financial results. ...
(Date:4/30/2015)... April 30, 2015 Nitto Avecia, ... announces its increased capacity and production capability to ... client variety and service fulfillment continues to drive ... improve process throughput capacities. , In ... installed downstream process equipment at its Milford, MA ...
(Date:4/30/2015)... Paul G. Allen Family Foundation announced today the award ... of researchers with projects at the frontier of one ... human brain cells in the laboratory. The projects are ... years.  "This new cohort of Allen ... the field of neuronal maturation is at the leading ...
Breaking Biology Technology:AMRI to Present at the Bank of America Merrill Lynch 2015 Health Care Conference 2Roka Bioscience to Report First Quarter 2015 Financial Results On May 7, 2015 2Biotechnology Manufacturer Nitto Avecia Expands Capacity to Meet Client Demand 2The Paul G. Allen Family Foundation Awards $7.5 Million To Study Brain Cell Growth And Development 2The Paul G. Allen Family Foundation Awards $7.5 Million To Study Brain Cell Growth And Development 3The Paul G. Allen Family Foundation Awards $7.5 Million To Study Brain Cell Growth And Development 4The Paul G. Allen Family Foundation Awards $7.5 Million To Study Brain Cell Growth And Development 5
... a technology that allows researchers to test new drugs on ... fortunately for one local company, the federal government is willing ... , ,To improve its technology, InvivoSciences, LLC , a ... National Center for Complementary and Alternative Medicine at ...
... Inc. has reached the public sector with the sale ... Stratton VA Medical Center in Albany, N.Y., a ... now is treating patients with TomoTherapy's cancer-targeting system, the company ... what the company calls conformal radiation therapy that targets tumors ...
... -You're in an elevator next to a venture capitalist who ... first thing about it yet. , ,You have 90 ... the Elevator Pitch Olympics, a yearly event at the ... investors dished out advice and a few hard knocks to ...
Cached Biology Technology:InvivoSciences receives NIH grant for heart technology 29 tips for making 90 seconds count in an investment pitch 2
(Date:4/17/2015)... 2015 Increasing adoption ... advancement to drive biometric systems market in ... According to a recently released TechSci ... ", biometric systems market in Saudi ... at CAGR of over 22% through 2020. The ...
(Date:4/13/2015)... , April 13, 2015 ... Research, "Global Biometrics Market Forecast & Opportunities, 2020", the ... CAGR of around 14% till 2020. The biometrics ... large scale implementation and review of biometric management ... of new products with greater efficiency, are resulting ...
(Date:4/9/2015)... JOSE, Calif. , April 9, 2015 ... human interface solutions, announced today that it will report ... on Thursday, April 23, 2015, after the close of ... for analysts and investors at 2:00 p.m. PT (5:00 ...   To participate on the live call, ...
Breaking Biology News(10 mins):Saudi Arabia Biometric Systems Market to Grow at 22% Through 2020, Says TechSci Research 2Saudi Arabia Biometric Systems Market to Grow at 22% Through 2020, Says TechSci Research 3Global Biometrics Market to Cross US$ 21 Billion by 2020: TechSci Research 2Synaptics to Report Third Quarter Results on April 23 2
... the U.S. should spend roughly $197 million more than it ... public health. The analysis found that the U.S. spends ... the health impacts of climate change, says Marie S. O,Neill, ... adequately address the public health issues related to global warming, ...
... aimed at developing drugs that stop malaria parasites from ... from the Walter and Eliza Hall Institute, named a ... and Science. Dr Baum, a senior postdoctoral fellow ... the molecular mechanisms that control the malaria parasite,s movement. ...
... porpoisesfirst dipped their toes into water, a series of evolutionary ... the larger hoofed animal group. But what happened first, a ... the loss of their ability to walk? A new paper ... debate using a massive data set of the morphology, behavior, ...
Cached Biology News:US needs nearly $200 million more on climate-related health research 2Malaria research wins Jake Baum a Young Tall Poppy award 2Getting a leg up on whale and dolphin evolution 2Getting a leg up on whale and dolphin evolution 3
Anti-human L-Ficolin, Clone GN4, Monoclonal Antibody...
14-3-3 tau Antibody Shipping Temperature: HOT Storage Temperature: -20C...
Applications: Western blotting Stability: Room temperature, indefinitely ...
... Rabbit Antibody to ARG. Vasopressin ... vasopression peptide. Cells are found in ... fibers are found throughout the hypothalmus ... perfused tissue. Immunogen: ...
Biology Products: