Ubiquitin and neurofilament expression in anterior horn cells in amyotrophic lateral sclerosis: possible clues to the pathogenesis

摘要: Cytoskeletal abnormalities are a prominent pathological feature of anterior horn cells in amyotrophic lateral sclerosis (ALS), and thought to be involved the process motor neuron death. Skein-like filamentous inclusions have been detected by immunocytochemical staining for ubiquitin, stress protein targeting abnormal proteins proteolysis. So far, identification target has elusive. We studied ultrastructural localization ubiquitin neurofilaments post-embedding immunogold staining. In skein-like arrays, strong labelling was concentrated on abnormally formed 15-20 nm filaments; neurofilament localized 10 filaments adjacent or continuity with filaments. addition, Bunina bodies were major site accumulation. Our results suggest that ubiquitinated might originate from aggregated proteins, which no longer recognized antibodies epitopes. Furthermore, presence suggests that, addition its protective role, directly implicated mechanism programmed neuronal death ALS.