Identified 3,500–5,000 transcripts in mouse and human stem cell-derived spinal motor axons, most of which are required for oxidative energy production and ribogenesis. Axons contained transcription factor mRNAs, e.g., Ybx1, with implications for local functions. In SOD1G93A mutant, identifying 121 ALS-dysregulated transcript, including Nrp1, Dbn1, and Nek1, a known ALS-causing gene. Here, DBN1 is linked to amyotrophic lateral sclerosis.