RNA quality control system goes awry in frontotemporal lobar degeneration

(Osaka University) Researchers from Osaka University have uncovered that the RNA exosome is critical for the degradation of defective repeat RNA derived fromC9orf72 repeat expansions in patients withC9orf72-associated frontotemporal lobar degeneration and amyotrophic lateral sclerosis. The repeat RNA and resultant toxic proteins impaired RNA exosome function, which led to further accumulation of the defective RNA and protein. The findings outline a cycle that may accelerate neurodegeneration. Therapies disrupting the cycle may help treat the disorder.

Source: EurekAlert! - Biology - August 24, 2020 Category: Biology Source Type: news