maybe hope

[ardalon]

Maybe hope !

Munich, 21 January 2014. Professor Dieter Edbauer (37) from the Munich office of the DZNE is given the "Consolidator Grant" of two million Euros from the European Research Council (ERC). The award is given to young researchers who are entering unknown territory with their projects. With the financial support Edbauer want to investigate hitherto incurable brain diseases and develop measures for their treatment. It is about the most common hereditary variant of amyotrophic lateral sclerosis (ALS) and frontal-temporal dementia (FTD). These diseases overlap in their causes and symptoms. They may be associated with dementia, personality changes and also with voice and movement disorders. Often the patients die within a few years.
the link:
DZNE: Ansprechpartner
Arda

 

[ardalon]

sorry it seems as if the link didn't work,
here is the article:

The molecular biologist is focusing on extremely unusual proteins, which form clumps in the brains of patients. It is just a few months since Edbauer and his colleagues deciphered the composition and formation of these proteins.

“The studied proteins consist of a chain of identical elements. We call them dipeptide-repeat proteins, DPRs for short. They normally do not occur in the body, which means they are very unusual,” explains Edbauer, who leads a research group at the DZNE’s Munich site.

Translation errors in the brain
The researchers were able to trace these proteins back to a genetic peculiarity. “The DNA of these patients contains a particular section, which repeats itself hundreds or even thousands of times. In healthy people, this sequence occurs less than 30 times. The formation of a protein product is highly unexpected, because these repetitive sequences are located in an area of the genome which normally is not translated into proteins,” says the molecular biologist.

The function of these proteins has not yet been determined. “We know very little about their effects and properties. Our project is breaking new scientific ground,” says Edbauer. “It seems that the DPRs are of no use whatsoever to the organism and that they damage nerve cells. We want to investigate this in depth. We also want to find out how these proteins are formed, because it is absolutely unclear why this particular area of the genome is translated at all.”

Targeted treatment
Besides investigating tissue samples from patients, Edbauer’s team will also examine cell cultures and genetically modified mice to find new targets for treatments. “We want to test substances which prevent the creation of these proteins or slow down the aggregation process,” is how he describes the research plan.

Currently available treatments for ALS and FTD can at best alleviate the symptoms. At present, there would be no way of stopping the progression of these diseases, explains Edbauer: “The DPRs could be ideal targets for a specific treatment, because they do not occur in healthy people. If we devise a treatment, which specifically tackles these proteins, we should be able to avoid the disruption of vital metabolic processes. This minimises the risk of side-effects.”

The Munich-based researcher sees the grant of the ERC as a great opportunity: “I hope we can take a major step towards developing a causative treatment against these catastrophic diseases.”

Arda