Abstract

Farnesylated prelamin A is a processing intermediate produced in the lamin A matura-tion pathway. Accumulation of a truncated far-nesylated prelamin A form, called progerin, is a hallmark of the severe premature ageing syn-drome, Hutchinson-Gilford progeria. Progerin elicits toxic effects in cells, leading to chro-matin damage and cellular senescence and ultimately causes skin and endothelial defects, bone resorption, lipodystrophy and accelerated ageing. Knowledge of the mechanism underly-ing prelamin A turnover is critical for the development of clinically effective protein inhibitors that can avoid accumulation to toxi

Similar works

This paper was published in CiteSeerX.

Having an issue?

Is data on this page outdated, violates copyrights or anything else? Report the problem now and we will take corresponding actions after reviewing your request.