Chemical Inhibition of NAT10 Corrects Defects of Laminopathic Cells

Remodelin Nuclear Defects

Deregulation of A-type lamin proteins leads to disorganization of chromatin structure and misshapen nuclei, which are believed to underlie the pathologies of various human diseases, including the premature aging disorder Hutchinson Gilford progeria syndrome (HGPS) and various cancers.

Larrieu
et al.

(p.
527
) developed a small molecule, Remodelin, that not only improved nuclear shape defects of human lamin A/C–depleted cells, HGPS cells, and aged normal cells, but also decreased the levels of a DNA damage marker and improved global cellular fitness.