CXCR4 undergoes early autocrine and proteostatic deregulation, and intracellular sequestration and aggregation as a result of Ranbp2 loss in mouse Thy1 motoneurons which causes ALS syndromes with hypoactivity followed by hindlimb paralysis, respiratory distress, and, ultimately, death [46]. The gene discussed is CXCR4; the disease is amyotrophic lateral sclerosis.