Mutant TDP-43 and FUS (mTDP-43 and mFUS) are found in cytoplasmic inclusions in the disease state while the accumulation of wild type TDP-43 and FUS (wtTDP-43 and wtFUS) are observed in an increasing number of disorders including Alzheimer's Disease, Parkinson's Disease and the polyglutamine diseases (reviewed in [10]). The gene discussed is FUS; the disease is early-onset autosomal dominant Alzheimer disease.