In July, the Beatson International Cancer Conference took place at The Beatson Institute for Cancer Research in Glasgow, UK. The Beatson Institute is a Cancer Research UK-funded centre which focuses on understanding cancer cell behaviour and developing new therapies and diagnostic tools. A wide variety of research is carried out at the institute, such as the work by Frezza et al. (2011), which was recently discussed in our blog. This meeting comprised of talks from both academic and industry professionals. For example, Dr Saul Rosenberg from Abbott Laboratories spoke about the development of the peptide mimetic ABT-737, which has been shown by Cash et al. (2011) to be effective in inducing apoptosis in FLCN-/- cells. Professor Norbert Perrimon from Harvard Medical School also spoke about using genome-wide RNAi screens and TAP-tag pull-down assays to understand how signalling pathways in cells overlap. These techniques could also be used to help untangle the signalling networks involved in BHD syndrome.
More recently in September, the International Tuberous Sclerosis Complex (TSC) Research Conference was hosted by the Tuberous Sclerosis Association in Belfast, UK. Of particular interest were two talks which specifically focussed on BHD syndrome. The first, by Deirdre Donnelly of the TS Clinic in Belfast, introduced BHD to the audience and gave an overview of its clinical similarities and differences with TSC. The second by Dr Andrew Tee of Cardiff University discussed the dysregulation of HIF signalling, which is observed in both TSC and BHD syndrome. To learn more about Dr Tee’s BHD research, click here to be directed to his lab-profile from a previous blog entry.
Information gathered at this meeting was further supplemented by talks at the Second French scientific day on TSC in Paris in October. This study day was jointly organised by the Association Sclérose Tubéreuse de Bourneville and the French rare epilepsies and TSC reference centre at the Hôpital Necker, Paris. Here, the promising results of a number of clinical trials were presented by Professor David Franz from Cincinnati Children’s Hospital. These trials are largely based on the mTORC1 inhibitors rapamycin and everolimus, and they are thought to work through a pathway which is illustrated in our FLCN-associated signalling diagram.
- Cash TP, Gruber JJ, Hartman TR, Henske EP, & Simon MC (2011). Loss of the Birt-Hogg-Dubé tumor suppressor results in apoptotic resistance due to aberrant TGFβ-mediated transcription. Oncogene, 30 (22), 2534-46 PMID: 21258407
- Frezza C, Zheng L, Folger O, Rajagopalan KN, MacKenzie ED, Jerby L, Micaroni M, Chaneton B, Adam J, Hedley A, Kalna G, Tomlinson IP, Pollard PJ, Watson DG, Deberardinis RJ, Shlomi T, Ruppin E, & Gottlieb E (2011). Haem oxygenase is synthetically lethal with the tumour suppressor fumarate hydratase. Nature, 477 (7363), 225-8 PMID: 21849978