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Slow motor neurons resist pathological TDP-43 and mediate motor recovery in the rNLS8 model of amyotrophic lateral sclerosis by Seong Kwon Hur; Mandana Hunter; Myrna A. Dominique; Madona Farag; Dejania Cotton-Samuel; Tahiyana Khan; John Q. Trojanowski; Krista J. Spiller; Virginia M.-Y. Lee is a Medicine article available to read on EtoBox.
What is Slow motor neurons resist pathological TDP-43 and mediate motor recovery in the rNLS8 model of amyotrophic lateral sclerosis about?
In the intermediate stages of amyotrophic lateral sclerosis (ALS), surviving motor neurons (MNs) that show intrinsic resistance to TDP-43 proteinopathy can partially compensate for the loss of their more disease-susceptible counterparts. Elucidating the mechanisms of this compensation may reveal approaches for attenuating motor impairment in ALS patients. In the rNLS8 mouse model of ALS-like pathology driven by doxycycline-regulated neuronal expression of human TDP-43 lacking a nuclear localization signal (hTDP-43ΔNLS), slow MNs are more resistant to disease than fast-fatigable (FF) MNs and can mediate recovery following transgene suppression. In the present study, we used a viral tracing strategy to show that these disease-resistant slow MNs sprout to reinnervate motor endplates of adjacent muscle fibers vacated by degenerated FF MNs. Moreover, we found that neuromuscular junctions within fast-twitch skeletal muscle (tibialis anterior, TA) reinnervated by SK3-positive slow MNs acquire resistance to axonal dieback when challenged with a second course of hTDP-43ΔNLS pathology. The selective resistance of reinnervated neuromuscular junctions was specifically induced by the unique pat
Who reads Slow motor neurons resist pathological TDP-43 and mediate motor recovery in the rNLS8 model of amyotrophic lateral sclerosis?
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- Author
- Seong Kwon Hur; Mandana Hunter; Myrna A. Dominique; Madona Farag; Dejania Cotton-Samuel; Tahiyana Khan; John Q. Trojanowski; Krista J. Spiller; Virginia M.-Y. Lee
- Publisher
- Springer Science and Business Media LLC
- Published
- 2022
- Language
- EN
- Field
- Medicine (Health Sciences)