Acta Neuropathologica (2018) 135:475–479 https://doi.org/10.1007/s00401-018-1823-1 COMMENTAR Y 1,2,3 1,4 1,4 1,4 1,4 Thomas Arzberger · Martin H. Schludi · Carina Lehmer · Bettina Schmid · Dieter Edbauer Received: 10 February 2018 / Accepted: 10 February 2018 / Published online: 15 February 2018 © The Author(s) 2018. This article is an open access publication A (G4C2) expansion with several hundred or thousand hippocampus, thalamus and cerebellum, but scarce in brain repeats in the first intron upstream of the C9orf72 coding stem and spinal cord. Although the DPR proteins co-aggre- region is the most common cause of amyotrophic lateral gate predominantly in cytoplasmic and less frequently in sclerosis (ALS) and frontotemporal lobar degeneration intranuclear inclusions in neurons, the individual proteins (FTLD), but the driver mechanism remains unclear . have very different biophysical properties. Cryoelectron Three main pathomechanisms have been proposed, but their tomography shows that poly-GA forms twisted ribbons that relative role is vigorously debated because they require par- interfere with proteasome function . Poly-GR and -PR tially opposing therapeutic strategies. Three reports in this undergo liquid–liquid phase separation in vitro and interfere issue take a provocative stance strongly arguing for either with the dynamics of the nucleolus and stress granules  RNA or protein toxicity in C9orf72 pathogenesis
Acta Neuropathologica – Springer Journals
Published: Feb 15, 2018
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