Several diseases involving a variety of cells and tissues are associated with defective enzymes of the Golgi apparatus (GA). An intact GA of neurons is crucial for the physiological function of axons and presynaptic terminals since proteins destined for fast axoplasmic transport are processed by the organelle. Despite the obvious importance of the GA of neurons, its function and involvement in pathological reactions have not been studied systematically. The purpose of this paper is to draw attention to the contribution of the neuronal GA in pathology using two paradigms: (1) the involvement of the neuronal GA in the pathogeneses of amyotrophic lateral sclerosis (ALS), in an animal model of ALS, and in Alzheimer's disease; and (2) the elucidation of a retrograde transport pathway involving the neuronal transgolgi network, in vitro and in vivo, and the participation of this pathway in the toxicity and/or endocytosis of ricin and other toxic or non-toxic ligands.