One of the biggest challenges currently facing BHD researchers is elucidating the role of FLCN’s within the cell. There’s no real way of fixing something unless you know how everything should normally fit into place, whether it be a bike, a car or even a human!
A new article in the journal Molecular Cancer, by Seung-Beom Hong et al1 for me, is something to get quite excited about and I think that a lot of future research will look to this as it’s starting point.
Using an elegant combination of in vitro cell based assays, in vitro xenograft studies and gene expression profiles, the authors have successfully demonstrated that FLCN has an essential role in the regulation of components of TGF-β signalling and indeed confirm their deregulation in BHD-associated kidney tumours. This is a great step forward in understanding pathogenic mechanism underlying BHD syndrome and as such, throws up many new avenues for research that I’m sure will be followed up.
Regular readers will know that I usually dissect published information and try to look for synergy with other work in the area, and I’ll be doing so with all the data that this paper has generated in due course – I just thought I’d give everyone a heads up about its publication and why it’s so exciting.
1. Hong SB, Oh H, Valera VA, Stull J, Ngo DT, Baba M, Merino MJ, Linehan WM, Schmidt LS. Tumor suppressor FLCN inhibits tumorigenesis of a FLCN-null renal cancer cell line and regulates expression of key molecules in TGF-beta signaling. Mol Cancer. 2010 Jun 23;9(1):160.
www.BHDsyndrome.org – the primary online reference site for anyone interested in BHD Syndrome