A rare gene defect may trigger some forms of inherited motor neuron disease (MND), scientists have discovered.The genetic mutation was found in a large family affected by amyotrophic lateral sclerosis (ALS).The disease belongs to a group of MND disorders that cause the progressive destruction of motor nerves, leading to severe disability and death. A celebrated sufferer of ALS is the renowned physicist Professor Stephen Hawking.Previous research has shown that a protein called TDP-43 accumulates in the nerve cells of people with ALS, but not unaffected individuals.
