The comprehensive FUS gene content is maintained at the canonical gene page:
This page provides an in-depth overview of:
- Gene structure and protein: FUS encodes a 526-amino-acid RNA-binding protein with multiple functional domains (RGG domains, zinc finger, NLS, PY motif)
- Physiological functions: RNA processing, splicing regulation, transcription, DNA damage response, nucleocytoplasmic transport
- Role in ALS/FTD: ~5% of familial ALS caused by FUS mutations (p.R521C, p.P525L), cytoplasmic FUS inclusions, nuclear import defects, liquid-liquid phase separation
- Mechanisms of toxicity: gain-of-function (aggregation, nuclear loss), RNA granule dysregulation, stress granule persistence
- Animal models: FUS transgenic mice and knockout models recapitulate key features of ALS pathology
FUS (Fused in Sarcoma, also called TLS) is an RNA-binding protein that aggregates in ALS and FTD. Like TDP-43, FUS pathology is found in a significant subset of ALS and FTD cases. The canonical gene page at /genes/fus contains detailed references.