Pietro Fratta

Very excited to share our latest preprint! We looked at loss and gain of function, innervation, reinnervation, and in vivo axonal transport in a FUS-ALS mouse model!

www.biorxiv.org/content/10.1...

@uclqsion.bsky.social @beccasimkin.bsky.social @andrewtosolini.bsky.social @frattalab.bsky.social

A 🧵 to walk those interested through this paper...

Our new review on the challenges of modelling TDP-43 pathology in mice is just out:
rdcu.be/ejZ5a
As usual, together with Lizzy Fisher. Thanks to Jose Brito and @lucastaoro.bsky.social from the lab for putting the data together for the comprehensive tables.

Our work focussed on selected lower limb, upper limb and bulbar regions, but whole body MRI is the next step.

There are limitations and breathing difficulties make tolerating an MRI scan problematic when ALS progresses. But shorter protocols and alternative positioning may overcome this.

Muscle MRI quantifies disease progression in amyotrophic lateral sclerosis Background and objectives Quantitative and operator-independent biomarkers of disease progression are urgently needed in amyotrophic lateral sclerosis (ALS) research. We assess the potential of skelet...

Something different from the lab!
We need better ALS biomarkers to minimise size, duration and cost of trials. Muscle MRI could work as it reliably detects progressive atrophy, and correlates with muscle strength loss.
@uclqsion.bsky.social @uclbrainscience.bsky.social
jnnp.bmj.com/content/earl...

Seeded aggregation of TDP-43 induces its loss of function and reveals early pathological signatures TDP-43 pathology characterizes neurodegenerative diseases. Scialò et al. use in vitro-generated and patient-derived TDP-43 aggregates as seeds to show that seed-induced TDP-43 cytoplasmic aggregation ...

Happy to share our new study that was just published in Neuron! www.cell.com/neuron/fullt...