RHOT1 and amyotrophic lateral sclerosis: The study further revealed a specific requirement for Miro1 in upper motor neuron development and post-mitotic function, with targeted disruption of Miro1 within the cerebral cortex that caused retrograde mitochondrial motility defects in cortical neurons, depletion of mitochondria from neuronal axons within the corticospinal tract, and progressive upper-body ALS.