C9orf72 poly(PR) aggregation in nucleus induces ALS/FTD-related neurodegeneration in cynomolgus monkeys.

Poly(PR) is a dipeptide repeat protein comprising proline and arginine residues. It is one of the translational product of expanded G4C2 repeats in the C9orf72 gene, and its accumulation is contributing to the neuropathogenesis of C9orf72-associated amyotrophic lateral sclerosis and/or frontotemporal dementia (C9-ALS/FTD). In this study, we demonstrate that poly(PR) protein alone is sufficient to induce neurodegeneration related to ALS/FTD in cynomolgus monkeys.

Effective removal of nanoplastics from water by cellulose/MgAl layered double hydroxides composite beads.

Micro/nanoplastic pollution is an emerging concern all over the world as it has a certain impact on the eco-environment and human health. In this study, cellulose/MgAl layered double hydroxides (LDHs) composite beads were prepared for the removal of polystyrene nanoparticles by utilizing the porous properties of cellulose and the unique positive charge of LDHs. The effects of pH, contact time, initial concentration, temperature, humic acid, and ionic strength on the attachment of nanoplastics were studied.

Generation of a Hutchinson-Gilford progeria syndrome monkey model by base editing.

Many human genetic diseases, including Hutchinson-Gilford progeria syndrome (HGPS), are caused by single point mutations. HGPS is a rare disorder that causes premature aging and is usually caused by a de novo point mutation in the LMNA gene. Base editors (BEs) composed of a cytidine deaminase fused to CRISPR/Cas9 nickase are highly efficient at inducing C to T base conversions in a programmable manner and can be used to generate animal disease models with single amino-acid substitutions.