Progerin is a truncated version of lamin A protein involved in Hutchinson-Gilford progeria syndrome. Progerin is most often generated by mutation in the lamin A gene, LMNA. This mutation gives rise to a form of lamin A with a deletion of 50 amino acids near the C-terminus. Lamin A constitutes a major structural component of the lamina, a scaffold of proteins found inside the nuclear membrane of a cell; progerin can not properly integrate into the lamina, which disrupts the scaffold structure and leads to significant disfigurement of the nucleus, characterized by a lobular shape. This interferences with consistency of the nuclear envelope can result in misshapen cell nuclei. The consequence of this LMNA mutation leading to excessive accumulation of progerin which in turn result is the rare premature aging genetic disorder called Hutchinson-Gilford progeria syndrome or simply Progeria.

Recently progerin is implied in the normal aging process as well. The progerin accumulation while we age was attributed to the cause of normal aging process. It has been found that progerin is generated through aging. A Swedish research team has evaluated the inducement of progeria gene and found that as people age more and more “progerin RNA is produced”. It has been concluded that accumulation of progerin in typical cells could cause similar effects to HGPS patients in elder people. In normal human beings, the increase in progerin in the body was reportedly 3.3 percent every year, that in progeria patients it was much higher. This study has convinced researchers of a definite link between progerin and aging who are now trying to establish its connection with the heart disease.

In addition, progerin activates genes that regulate stem cell differentiation via the Notch signaling pathway. which means progerin accumulation could interfere our natural repair and regenerative mechanism and which in turn could cause accumulation of misrepair or unable to repair and regenerate damaged cells and tissues.