Neuromuscular junction denervation and terminal Schwann cell loss in the hTDP-43 overexpression mouse model of amyotrophic lateral sclerosis (ALS).

Abrar AlhindiMegan ShandHannah L SmithAna S LeiteYu-Ting HuangDinja van der HoornZara RidgwayKiterie M E FallerRoss A JonesThomas H GillingwaterHelena Chaytow

Published in: Neuropathology and applied neurobiology (2023)

mice represent a severe model of ALS, with NMJ pathology/denervation of distal muscles and motor neuron loss, as observed in ALS patients. This model therefore provides an ideal platform to investigate mechanisms of dying back pathology, as well as NMJ-targeting disease-modifying therapies in ALS.

Keyphrases

amyotrophic lateral sclerosis
mouse model
end stage renal disease
newly diagnosed
single cell
cell proliferation
prognostic factors
minimally invasive
high throughput
stem cells
cancer therapy
mesenchymal stem cells
high fat diet induced
wild type