Drag nuclear membrane nodes to test structural stiffness
Cell Line / Type
Progerin Level0.85
Mechanical Stress0.50
FTI Dosage (Lonafarnib)0 mg
LMNA Mutation Rate1.00
Nuclear MorphometryPATHOLOGY
Circularity Index0.42
Blebbing Frequency0.78
DNA Damage Foci Count18
FTI Rescue Efficiency0%
Senescence OnsetDay 14
Molecular Mechanism
Classic HGPS is driven by the c.1824C>T mutation in the LMNA gene, activating a cryptic splice site. This produces truncated Progerin which retains a toxic farnesyl lipid anchor, permanently embedding into the nuclear lamina and destabilizing membrane mechanics.