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Neurology

New Clue to Causes of Neurodegenerative Diseases, Implications for Motor-Neuron & Huntington's Disea

12 years, 5 months ago

250  0
Posted on Jul 06, 2005, 8 a.m. By Bill Freeman

Understanding of biochemical mechanisms enables faster research paths to targeted, effective therapies. Medical News Today notes progress in knowledge of mechanisms of a range of age-related neurodegenerative conditions: "Many of these diseases are characterised by accumulation, inside nerve cells, of clumps of toxic proteins. ... failure of the dynein system causes the degeneration in a form of motor neuron disease, and that it is also involved in other conditions such as Huntington's disease.
Scientists funded by the Medical Research Council (MRC), the Wellcome Trust, BBSRC and the EU have made an important discovery about the mechanisms underlying degenerative brain diseases. The findings, published in this week's edition of the science journal Nature Genetics, may have implications for therapeutic strategies for some forms of motor neuron disease, Huntington's disease and Alzheimer's.

Late-onset neurodegenerative diseases are a major health burden on the population, yet little is known about the chemical changes that trigger the degeneration of nerve cells. However, many of these diseases are characterised by accumulation, inside nerve cells, of clumps of toxic proteins. The new research - carried out at the Cambridge Institute for Medical Research, the MRC Mammalian Genetics Unit at Harwell and the Department of Genetics, University of Cambridge - has concentrated on tiny molecular motors called dyneins, which are known to be important for moving proteins around inside nerve cells. The researchers found that dyneins play a crucial role in the delivery of toxic proteins to the waste disposal units of cells. When dyneins are defective or absent, this waste disposal system is stalled, clumps of proteins appear, and cell function is compromised.

The study found that failure of the dynein system causes the degeneration in a form of motor neuron disease, and that it is also involved in other conditions such as Huntington's disease. It also provided further evidence for the idea, pioneered by the same group of scientists, that a key factor in the severity of these diseases is the rate at which these toxic clumps of protein can be removed. Enhancing the degradation of these protein clumps has the potential to delay the onset of, or even reverse, this group of diseases. This might be exploited in new approaches to prevention or therapy.

The lead scientist, David Rubinsztein of the Cambridge Institute for Medical Research, said:

"These findings provide us with real insight into the molecular basis of certain motor neuron diseases and the mechanisms by which toxic proteins accumulate and are broken down. They also contribute greatly to our understanding of possible therapeutic strategies for these diseases."

The Medical Research Council (MRC) is a national organisation funded by the UK tax-payer. Its business is medical research aimed at improving human health; everyone stands to benefit from the outputs. The research it supports and the scientists it trains meet the needs of the health services, the pharmaceutical and other health-related industries and the academic world. MRC has funded work which has led to some of the most significant discoveries and achievements in medicine in the UK. About half of the MRC's expenditure of more than £500 million is invested in its 40 Institutes, Units and Centres. The remaining half goes in the form of grant support and training awards to individuals and teams in universities and medical schools. Web site at: http://www.mrc.ac.uk.

The Cambridge Institute for Medical Research (CIMR) is a modern research facility in Cambridge, where clinical and basic science converge in the study of the molecular mechanisms of disease. All of the scientists in CIMR are members of 'home' Departments of the University of Cambridge. Presently, the 'home' Departments are Clinical Biochemistry, Haematology, Medicine, Medical Genetics, Oncology and Pathology. CIMR supports excellent basic research and encourages collaborations between research groups and across major research themes, which broadly encompass medical genetics, metabolic medicine, cell biology, immunology, developmental biology and structural biology. It is housed in the Wellcome Trust/MRC building and is established under regulations approved by the Faculty Board of Clinical Medicine, University of Cambridge. Some of the core scientific support in CIMR is provided by a five-year award from the Wellcome Trust as a 'strategic initiative in the clinical sciences at CIMR' cimr.cam.ac.uk/index.html

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