Faculty of Medical Sciences

Staff Profile

Dr Daniel Williamson

Newcastle University Research Fellow

Background

Daniel Williamson completed a BSc(Hons) Genetics at Nottingham University followed by a PhD and  Post-Doc studying the genetics of Rhabdomyosarcoma at The Institute of Cancer Research. He won a Marie-Curie fellowship from the European Commission to move to Institut Curie in Paris investigating childhood cancer genetics and gaining expertise in Bioinformatics and genomics analysis. He was appointed Lecturer in Paediatric Neuro-Oncology at the Northern Institute for Cancer Research at Newcastle University in 2010.

Research

Malignant Rhabdoid Tumours (MRT) are highly aggressive brain tumours which occur primarily in infants and have an extremely poor prognosis (15-30% survival). Rhabdoid tumours may occur in any part of the body and share a common pathology and biology (ATRT is the term for rhabdoid tumours of the CNS).

The genetic cause of rhabdoid tumours is well-established and unusually singular; biallelic inactivation of SMARCB1,  a subunit of the SWI/SNF chromatin remodelling complex, is sufficient to cause >90% of ATRT.

Re-expressing a functional copy of SMARCB1  in MRT cells induces growth arrest and differentiation, effectively causing MRT cells to revert to normal. There are no other recurrent mutations or copy number changes; in some instances SMARCB1  loss is the sole mutational change . The primacy of SMARCB1  mutation offers a clear route to targeted therapies, however we lack the mechanistic knowledge to rationally select effective biological targets for therapeutic intervention. Since 2012, we have led a unique CCLG Biological study, which implements a co-ordinated approach to ATRT translational research, allowing us to collect and profile retrospective and prospective cohorts from the U.K.

Publications