Abstract: Motor neuron diseases such as amyotrophic lateral sclerosis (ALS) remain largely incurable, with limited therapeutic options and only modest clinical benefits from currently approved drugs. Experimental models have been instrumental in shaping our understanding of disease mechanisms, yet translation into effective therapies has been challenging. Among these models, the Wobbler mouse, although less frequently employed than superoxide dismutase 1 (SOD1) transgenics, offers unique insights into motor neuron degeneration driven by disrupted intracellular trafficking and metabolic stress.