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Unravelling the secrets of ‘Huntingtin’ Protein - towards the treatment of ‘Huntingtons’ Disase

Huntingtons disease is an inherited disease of the nervous system due to the progressive gradual deterioration of the same, initially causing symptoms such as involuntary movements and balance problems and later on leading to declination of functions of speech as well.

All these days, until now, it was thought that this disease is due to a mutated form of the protein 'huntingtin' which is responsible for destroying the brain tissue.

Researchers have believed the mutation somehow caused the protein to be toxic. But until now, they have not been able to identify the function of the "normal" huntingtin protein present in people without the disease.

Researchers have uncovered the function of a protein that plays an important role in Huntington's disease (HD). Dr. Elena Cattaneo, from the University of Milan, in Italy and colleagues found that the huntingtin protein controls the production of another protein called brain-derived neurotrophic factor (BDNF), which is essential for maintaining the type of brain cells that die off in patients with Huntington's. It is this patients' mutated huntingtin protein that cannot produce proper amounts of BDNF, which leads to brain deterioration.

"Prior to this we thought that [Huntington's] was due to a toxic activity caused by the mutant protein. Here we show that there is a loss of normal function" Dr. Elena Cattaneo said. "There must be a cure for this disease," she added, "and there must be one very soon."

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