The optineurin-mediated autophagy-dysfunction by either overstimulation or by defects that block its function has attracted considerable attention due to its involvement in a variety of neurodegenerative diseases (glaucoma, amyotrophic lateral sclerosis, Parkinson’s disease, Huntington’s disease), inflammatory diseases (Crohn’s disease, acute liver failure, rheumatoid arthritis) cancer, and nephropathy (104). The gene discussed is OPTN; the disease is juvenile Huntington disease.