Bonn, April 6th, 2011. The German Centre for Neurodegenerative Diseases (DZNE) is an attractive address for top scientists from around the world – 53% of the scientific appointments at the DZNE in Bonn are of international origin. Also the US-American Walker Jackson will now establish a research group at the DZNE in Bonn. Jackson holds a Ph.D. from the University of Alabama-Birmingham. He did his postdoctoral training and was then a research associate at the prestigious Whitehead Institute for Biomedical Research at Massachusetts Institute of Technology. "When I visited the DZNE I was thrilled to find that everyone, from the support staff to the scientist to the leadership, are all excellent people, enthusiastic about the mission of the DZNE," says Jackson
Jackson's research interest is in prion proteins. In their normal form these proteins have a protective function in the brain. But if they fold incorrectly and adopt a specific three-dimensional structure, they turn into dangerous pathogens, which are deposited in the brain in the form of protein aggregates. Prions gained notoriety as a cause of "mad cow" disease in the 1980s, when the bovine epidemic spread in the UK, and then transferred into humans causing a new form of Creutzfeldt-Jakob disease.
Also in science, prions have raised much attention. They are the first proteins found to be infectious, and thus undermine a central dogma of molecular biology. Through his work at the Whitehead Institute, Walker Jackson has contributed significantly to this concept of protein infectivity: by genetically modifying mice, he showed that a small change in the prion protein is sufficient to cause a transmissible disease. If abnormal prion proteins come into contact with the body's normal form, the latter will be transformed into the pathological variant and form protein aggregates, which are typical for prion diseases.
The course of disease varies significantly between prion diseases. Fatal familial insomnia (FFI), for example, exhibit signs of sleep and autonomic nervous system dysfunction, whereas Creutzfeldt-Jakob disease (CJD) mainly affects mental and motor skills. Notably, the molecular mechanisms are very similar in both cases: The prion protein is altered and forms aggregates. With his research group in Bonn, Jackson will investigate why diseases that are so similar on the molecular level exhibit substantially different symptoms. In FFI, brain regions are affected that remain intact in CJD, and vice versa. Consequently, there are brain regions in both diseases that appear to have the ability to fight off the disease. "What protective mechanisms do the nerve cells use in these areas? If we gain a better understanding of this issue, this will be a good basis for the development of new therapies," says Jackson.
Age is a risk factor for many neurodegenerative diseases – at older age we are far more likely to suffer from Alzheimer's, Parkinson's or a prion disease. Through his research on fatal familial insomnia, Jackson came up with the idea that this correlation between age and susceptibility to neurodegenerative diseases has something to do with altered sleep patterns. "Sleep has many functions. Most likely, one important function is to repair cells that are damaged during the course of a day’s activity. It is possible that this repair mechanism is impaired in neurodegenerative diseases since we sleep less at older age and these are diseases that affect older people," says Jackson. The investigation of this hypothesis, using mouse models, is also within the scope of Jackson's research at the DZNE.
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Figure caption
Different brain areas are affected in mouse models of fatal familial insomnia and in Creutzfeldt-Jakob disease.
Contact:
Dr. Walker Jackson
German Center for Neurodegenerative Diseases (DZNE)
Sigmund-Freud-Str. 25
Building 344, BMZ 1
53175 Bonn
Phone: +49 (0) 228 / 28751704
Email: walker.jackson(at)dzne.de
Dr. Katrin Weigmann
German Center for Neurodegenerative Diseases (DZNE)
Press and Public Relations
Phone: +49 (0) 228 43302 /263
Mobile: +49 (0) 173 / 5471350
Email: katrin.weigmann(at)dzne.de
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