The progressive loss of structure and function of neurons is called neurodegeneration. As we grow older, the risk of neurodegeneration increases and we may develop the symptoms of neurodegenerative diseases.

 

What different kinds of neurodegenerative diseases are there?
There are a multitude of different neurodegenerative diseases. Some types are mainly characterised by cognitive impairment (dementia) such as loss of memory, disorientation and difficulty in managing the routine of everyday life. Alzheimer’s disease is the most common form of dementia with mainly cognitive symptoms, but there are also others like frontotemporal dementia and Creutzfeldt-Jakob disease, the human variant of ‘Mad cow disease’. Progressive tremors are the main symptom of Parkinson’s disease, which is one of the neurodegenerative diseases that results mainly in motor impairment (ataxia). Other important examples are Multiple Sclerosis and Amyotropic Lateral Sclerosis. The latter has become known because of  Stephen Hawking, a famous astrophysicist, who has been afflicted with ALS since 1963.


What causes neurodegeneration?
Although the symptoms of neurodegenerative diseases vary greatly, many have one distinct feature in common: the accumulation of protein clumps, so-called aggregates, inside or outside of neurons. What is yet unclear, however, is whether these protein aggregates cause neurodegeneration or whether they are a by-product of other biochemical processes?


ToPAG: a new interdisciplinary research project
To answer this question and to understand the processes involved in neurodegeneration, four leading scientists from the Max Planck Institute of Biochemistry and the Max Planck Institute of Neurobiology in Martinsried near Munich, Germany, have just endeavoured on a pioneering research project called ToPAG (Toxic Protein AGgregation in Neurodegeneration).
ToPAG will be funded by a six-year ERC synergy grant awarded by the EU. The researchers will focus on the basic cellular mechanisms of protein aggregate toxicity and establish mouse models to elucidate the role of protein aggregates in neurodegeneration from several angles using complementary methods.
“By identifying potential drug targets, this information may serve as the basis for developing urgently needed therapies that can halt or reverse disease processes of neurodegeneration”, says Prof. Ulrich Hartl, the lead researcher of the ToPAG research project.


Series on Alzheimer's disease
This In Focus article is the second in a series on Alzheimer's disease and dementia in general, published in September 2013, World Alzheimer’s Month.
Visit www.HorizonHealth.eu for updates on the latest dementia-related research in the EU.