Cell content effects toxicity of Huntington’s disease protein

The content of the cell contained in the protein mutation that leads to Huntington’s disease determines the toxicity of the protein and the severity of symptoms, according to a new study.

Huntington’s disease progressively breaks down nerve cells in the brain and is caused by a mutation in the Huntingtin gene that causes the huntingtin protein to possess an extended region containing glutamine.

The factors that affect the severity of a patient’s experiences of the disease have thus far remained a mystery.

It had been thought that the size of the extended region may have increased intensity of symptoms. However, research found that those with larger regions often experienced milder symptoms than those with smaller.

Scientists at the Georgia Institute of Technology in the US now believe that they have now shed some light on possible new ways to treat the disorder by identifying that the different prions found in the mutation effect the toxicity of the protein and severity of symptoms.

While findings must be subject to further testing, if confirmed they will enable doctors to better counteract the progression of Huntington’s disease.

Find out how Barchester works to create safe and understanding environments for those living with Huntington's disease.