FUS-R521C line 3313 expresses a FLAG-tagged, autosomal dominant mutant isoform of fused in sarcoma protein directed to brain and spinal cord (CNS neurons and astrocytes) by the Syrian hamster prion protein promoter. The hFUS*R521C mutation in the nuclear-localization domain is associated with familial amyotrophic lateral sclerosis (FALS). These mice may be useful in studying neurodegenerative disorders such as FALS (Lou Gehrig's Disease).
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