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The role of axonal RNA alterations in ALS
Aim: to address the hypothesis that early changes in axonal biology, and particularly in RNA localisation and axonal signalling, play a role in ALS.
Due to their unique morphology, axons represent >95% of MN mass and play a crucial role in neuronal function. Crucially, they are affected very early in the course of ALS.
The delivery of RNAs to axons is important for their development, maintenance and response to injury.
Microfluidic chambers (developed in the Schiavo Lab) allow the physical separation of motor axons from cell bodies. We use this system to study the RNA content and dynamics in motor axons.
We are using a novel mouse model of ALS carrying mutations in FUS (Lizzy Fisher and Anny Devoy) as a paradigm of disease.
Our investigation techniques include FISH, RNAseq, live axonal transport imaging.