Authors- Christopher P Webster, Emma F Smith, Claudia S Bauer, Annekathrin Moller, Guillaume M, Hautbergue, Laura Ferraiuolo, Monika A Myszczynska, Adrian Higginbottom, Matthew J Walsh, Alexander J Whitworth, Brian K Kaspar, Kathrin Meyer, Pamela J Shaw, Andrew J Grierson, & Kurt J De Vos,
A review by Dr Robert Hartley, CoolLED
This is a very interesting paper that uses LED illumination in all the fluorescence microscopy. They used both the pE-300 and pE-4000 from CoolLED in some of the experiments to produce beautiful images. I found this a particularly stimulating paper because before entering the commercial world a few years ago, I was involved in ALS research and know the research groups’ excellent work from the past. It was pleasing to see how the field has progressed in recent years with the identification of a gene responsible for 40% of familial ALS cases. In this paper they showed that this gene (C9orf72) plays a role in regulating Autophagy (essential for maintaining cell homeostasis). They observed that regulation of this pathway produced a pathology similar to that observed in cells from patients that have ALS.
The researchers presented their data in many ways and used many established techniques in a variety of cell types. In particular, they used fluorescence microscopy (and LED illumination) to quantify the puncta within cells that is indicative of in vivo data from patients. They use a combined approach of siRNA to reduce the level of the gene of interest (similar to what is observed in patients), allied with drug induced regulation of autophagy. Their results clearly show that C9orf72 is a regulator of autophagy initiation and that in patients, they suggest that autophagy may be the underlying cause of disease progression.
For further information on this research paper click here.