FUS and familial amyotrophic lateral sclerosis: Mutations in FUS are associated with familial amyotrophic lateral sclerosis patients.58 Disease-linked mutant FUS proteins preferentially associate with cytoplasmic stress granules.59 The FUSΔNLS mouse features engineering of the endogenous mouse Fus gene to incorporate a premature STOP codon, resulting in a C-terminally truncated NLS-deficient protein that accumulates in the cytoplasm.60