In this context, it is noteworthy that a missense mutation in the tubulin-specific chaperone E (Tbce) gene that causes motor neuron degeneration in the progressive motor neuronopathy (pmn) mouse strain, a model of human MND, causes microtubule loss similar to that induced by human ALS-linked TUBA4A mutations, and axonal transport defects (Bommel et al., 2002, Martin et al., 2002, Schäfer et al., 2016). Here, TBCE is linked to amyotrophic lateral sclerosis.