Peter Goodenough Memorial Lecture 2011: Major advances in our understanding of ALS

Description

Over the past 15 years, Professor Shaw has worked on the genetics and pathobiology of amyotrophic lateral sclerosis (ALS). His group was the first to discover mutations in the TDP-43 gene in familial and sporadic MND and demonstrated their toxicity in vivo. Although mutations are rare, they have provided a powerful biological tool to discover how TDP-43 is mislocalized and causes motor neuron degeneration.

Professor Shaw's group, in collaboration with the group of R.H Brown, contributed to the discovery of another ALS gene called FUS, which has very similar function to TDP-43. He and his colleagues are using cellular and animal models to elucidate the pathogenenic mechanisms underlying TDP-43 and FUS mislocalization and aggregation. They are also continuing the hunt for other ALS susceptibility genes using DNA capture and next generation sequencing.

Speaker

Christopher Shaw (MBChB, MD, FRACP, FRCP (Hon), FMedSci) is Professor of Neurology and Neurogenetics and Director of the MRC Centre for Neurodegeneration Research, King’s College London. He works as a consultant neurologist at King’s College Hospital where he runs a clinic for people with motor neuron disorders.