Mechanism behind neuron death in motor neurone disease and frontotemporal dementia discovered

Scientists have identified the molecular mechanism that leads to the death of neurons in amyotrophic lateral sclerosis (also known as ALS or motor neurone disease) and a common form of frontotemporal dementia.
A common characteristic of ALS and frontotemporal dementia is the build-up of clumps of misfolded RNA-binding proteins, including a protein called FUS, in the brain and spinal cord. This leads to the death of neurons, which stops them from communicating with each other and from reaching the muscles.In frontotemporal dementia, the abnormal gelling was found to be caused by defects in the chemical modification of FUS. In motor neuron disease, it was caused by mutations in the FUS protein itself which meant it was no longer able to change form. “This was a very exciting set of experiments where we were able to apply cutting edge tools from physics, chemistry and neurobiology to understand how the FUS protein normally works in nerve cells, and how it goes wrong in motor neurone disease and dementia. It now opens up a new avenue of work to use this knowledge to identify ways to prevent the abnormal gelling of FUS in motor neurone disease and dementia.”
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