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Scientists on track for early diagnosis of neurological diseases
July 01, 2003Possible treatments for fatal neurodegenerative diseases such as CJD, Alzheimer's, Parkinson's could result from University of Edinburgh research to find out how specific proteins cause deterioration in brain function. The scientists have discovered for the first time that protein 14-3-3 plays a key role in the development of certain brain diseases by binding to other brain proteins and causing them to malfunction. These findings, described by Professor Alastair Aitken, of the University of Edinburgh School of Biomedical Sciences in the journal Cell, could eventually lead to the development of gene therapy or drug treatments which would disable the harmful proteins.
It is already known that 14-3-3 appears in the spinal fluid of humans with Creutzfeldt-Jacob Disease and in animals with Bovine Spongiform Encephalopathy or scrapie. Members of the 14-3-3 protein family are involved in a range of other illnesses including Huntington's disease and related conditions. The latest discovery by the Edinburgh team and their American collaborators is that 14-3-3 proteins also play a crucial role in neurodegeneration in the disease Spinocerebellar Ataxia-Type 1, a progressive disorder affecting brain and limb function. This disease is caused by the production of an abnormally long stretch of the amino acid, glutamine, in a 'stuttering' effect in a protein called ataxin-1.
Professor Aitken explained: "A key feature of all these neurodegenerative diseases is the accumulation in specific areas of the brain of abnormal forms of proteins which results in degradation of brain function. The proteins that accumulate, due to their mis-folding or genetic mutation, are specific to each disease. However, the common feature of all these neurodegenerative diseases that is now emerging is the interaction of members of the 14-3-3 protein family with all of these proteins. 14-3-3 proteins interact with other proteins in cells to form protein complexes which are involved in cell growth, differentiation into different cell types, switching on or off of the expression of particular genes and the movement of proteins into different parts of the cell."
"If we can shed light on the mechanism of how these protein complexes form and how they function, then it may be possible to affect them in such a way as to be beneficial for patients. These findings also have implications for agriculture, where early diagnosis of BSE in cattle or scrapie in sheep could prevent contaminated animal products entering the food chain," said Professor Aitken. " This may lead to new treatments for disease and help fundamental research into how these protein complexes regulate brain functions."
Edinburgh, University of
"If we can shed light on the mechanism of how these protein complexes form and how they function, then it may be possible to affect them in such a way as to be beneficial for patients. These findings also have implications for agriculture, where early diagnosis of BSE in cattle or scrapie in sheep could prevent contaminated animal products entering the food chain," said Professor Aitken. " This may lead to new treatments for disease and help fundamental research into how these protein complexes regulate brain functions."
Edinburgh, University of
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