Hutchinson– Gilford Progeria Syndrome is a very rare disorder characterized by premature ageing caused due to mutation in LMNA gene. The child born with this disorder shows features of old age from first year of birth and generally dies in teenage. The clinical symptoms include alopecia, thin skin, stiffness of joints etc. All of the children suffering from this disease appear identical. The pa...

The relationship between progerias--diseases that resemble premature aging--and the normal aging process has been a source of debate in the aging research community. A recent study finds that LMNA, a gene targeted for mutation in Hutchinson Gilford Progeria Syndrome, may control the onset of aging-associated decline in normal fibroblasts.

Progerin, a mutated lamin A, causes the severe premature-aging syndrome Hutchinson-Gilford progeria (HGPS). Kubben et al. present a driving mechanism for HGPS involving trapping of NRF2 at the nuclear periphery by progerin. This local restriction results in impaired NRF2 signaling and chronic oxidative stress.