Muscle-specific Bet1L knockdown induces neuromuscular denervation, motor neuron degeneration, and motor dysfunction in a rat model of familial ALS

Muscle-specific Bet1L knockdown induces neuromuscular denervation, motor neuron degeneration, and motor dysfunction in a rat model of familial ALS

Amyotrophic lateral sclerosis (ALS) is a neuromuscular disease characterized by specific loss of motor neurons in the spinal cord and brain stem. Although ALS has historically been characterized as a motor neuron disease, there is evidence that motor neurons degenerate in a retrograde manner, beginn...

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Bibliographic Details
Main Authors: Adam Eckardt, Charles Marble, Bradley Fern, Henry Moritz, Charles Kotula, Jiayi Ke, Clarisse Rebancos, Samantha Robertson, Hiroshi Nishimune, Masatoshi Suzuki
Format: Article
Language:English
Published: Frontiers Media S.A. 2025-01-01
Series:Frontiers in Neuroscience
Subjects:
amyotrophic lateral sclerosis (ALS)
Bet1L
small interfering RNA (siRNA)
SOD1G93A rats
neuromuscular junction
skeletal muscle
Online Access:https://www.frontiersin.org/articles/10.3389/fnins.2025.1527181/full
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