The current understanding of motor neuron disease

Motor Neuron Disease is a term defining a group of neurodegenerative diseases, in which motor neurons degenerate, leading to muscle wasting, paralysis and ultimately death. Here I review the current understanding of Amyotrophic Lateral Sclerosis (ALS), the most common form of MND, looking particularly at the causative factors, and the available treatments for the disease. Familial ALS can be caused by a mutation in SOD1; research into the effects of this mutation has given an insight into the mechanisms by which MND progresses. Mutant SOD1 causes the initiation and progression of MND via protein aggregation, disrupted axonal transport, and oxidative stress within motor neurons. There is currently only one drug available for the treatment of motor neuron disease; Riluzole. I examine areas of current research into potential treatments for MND, in particular therapeutic gene silencing and the use of molecular chaperones to aid the folding of faulty SOD1 protein.