Using complementary proteomic and transcriptomic profiling, we found that ALS-linked UBQLN2 mutations (T487I and P497S) cause a robust elevation of the microtubule-associated protein MAP1B that was driven by an increase at the MAP1B transcript level and was not because of posttranslational mechanisms. The gene discussed is UBQLN2; the disease is amyotrophic lateral sclerosis.