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Lab Reports
June 4, 2014

Small Molecule Inhibits Defects of Premature Aging Disease

JAMA. 2014;311(21):2162. doi:10.1001/jama.2014.6402

A newly identified molecule named Remodelin can effectively treat the damaged nuclei of cells from patients with laminopathies, which include the premature-aging disorder Hutchinson-Gilford progeria syndrome, or HGPS (Larrieu D et al. Science. 2014;344[6183]:527-532).

Laminopathies stem from the down-regulation or mutation of the nuclear architecture proteins lamin A and C, which affect chromatin structure, gene expression, DNA replication, and DNA repair. Deregulation of lamin A expression or localization also occurs in various cancers.

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