Granules Gone Rogue: Nuclear and Cytoplasmic Ribonucleoprotein Structures in Amyotrophic Lateral Sclerosis-Fused in Sarcoma (ALS-FUS) Pathology - PubMed
8 hours ago
- #Fused in sarcoma
- #Ribonucleoprotein granules
- #Amyotrophic lateral sclerosis
- Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease causing motor neuron loss.
- Mutations in the FUS gene lead to aggressive ALS with early onset and rapid progression.
- FUS is a nuclear protein involved in DNA repair and RNA metabolism, forming ribonucleoprotein (RNP) granules.
- Pathogenic FUS mutations cause cytoplasmic mislocalization and aggregation, disrupting RNP granule dynamics.
- Mutant FUS alters stress granules and paraspeckles, increasing motor neuron vulnerability.
- FUS interacts with other ALS-associated proteins, suggesting a common disease mechanism.
- The review discusses FUS-mediated RNP granule regulation and potential therapeutic strategies.