In certain rare cases of ALS and FTLD the normally nuclear DNA/RNA-binding protein FUS is mutated, which causes FUS to mislocalize into cytoplasmic aggregates. Although several studies have suggested that this cytoplasmic mislocalization contributes to toxicity in ALS and FTLD, it was unclear if FUS’ RNA binding ability was also contributing to toxicity. Now, new research out of Dr. Udai Pandey’s laboratory at the Louisiana State University Health Sciences Center in New Orleans suggests that FUS’ ability to bind RNA is essential for FUS-associated neurotoxicity. The researchers generated a variety of FUS variants with combinations of ALS-relevant mutations and mutations in the RNA recognition motif (RRM) sequence. Drosophila carrying FUS variants with an ALS relevant mutation had brain morphology defects, motor neuron defects, and eye defects that were not observed in flies carrying FUS variants with the same ALS relevant mutation and additional mutations in the RRM sequence. These data suggest that the RNA binding ability of FUS might be essential for neurotoxicity. Click here to read the full story.
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