Cellular Pathobiology Section, Integrative Neuroscience Research Branch, Intramural Research Program, National Institute on Drug Abuse, NIH, 333 Cassell Drive, Baltimore, MD, 21224, USA.
Published: November 2020
AI Article Synopsis
Article Abstract
In a subgroup of patients with amyotrophic lateral sclerosis (ALS)/Frontotemporal dementia (FTD), the (G4C2)-RNA repeat expansion from C9orf72 chromosome binds to the Ran-activating protein (RanGAP) at the nuclear pore, resulting in nucleocytoplasmic transport deficit and accumulation of Ran in the cytosol. Here, we found that the sigma-1 receptor (Sig-1R), a molecular chaperone, reverses the pathological effects of (G4C2)-RNA repeats in cell lines and in Drosophila. The Sig-1R colocalizes with RanGAP and nuclear pore proteins (Nups) and stabilizes the latter. Interestingly, Sig-1Rs directly bind (G4C2)-RNA repeats. Overexpression of Sig-1Rs rescues, whereas the Sig-1R knockout exacerbates, the (G4C2)-RNA repeats-induced aberrant cytoplasmic accumulation of Ran. In Drosophila, Sig-1R (but not the Sig-1R-E102Q mutant) overexpression reverses eye necrosis, climbing deficit, and firing discharge caused by (G4C2)-RNA repeats. These results on a molecular chaperone at the nuclear pore suggest that Sig-1Rs may benefit patients with C9orf72 ALS/FTD by chaperoning the nuclear pore assembly and sponging away deleterious (G4C2)-RNA repeats.
Background: NUP155 was reported to involve breast invasive carcinoma and hepatocellular carcinoma. We hypothesized that NUP155 and NDC1impacted the progression of NSCLC.
Methods: The dataset was analyzed to find differentially expressed genes.
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Evaluation of the hydrogen storage capacity of porous rocks is crucial for underground hydrogen storage. Using H nuclear magnetic resonance (NMR) spectroscopy, we successfully characterized the hydrogen responses and identified storage mechanisms in Berea sandstone under varying water saturation. The results indicate that the injected hydrogen behaves as a free gas phase and is capable of occupying the empty pore volume regardless of the saturation state.
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