Progeria is a devastating disease in which the normal processes of aging appear to be alarmingly accelerated. Hutchinson-Gilford progeria is caused by a mutation in one of the nuclear lamin genes that leads to the production of a truncated form of lamin A (De Sandre-Giovannoli et al., Science 27 June 2003, p. 2055; published online 17 April 2003). Nuclear lamins line the inner nuclear membrane and help to maintain nuclear integrity. Cells taken from progeric patients display nuclear abnormalities, including severe morphological defects in the nuclear envelope. Now Scaffidi and Misteli show that simple expression of wild-type lamin does not rescue this cellular phenotype. Instead, suppressing the expression of the mutant lamin “cures” the nuclear envelope defects and concomitantly other defects, such as those in histone modification, are rescued—effectively reversing the cellular aging process. These findings may provide an avenue of hope for potential therapies aimed at this distressing, though extremely rare, condition. In addition, detailed understanding of the cellular aging process will be important in helping to combat the symptoms of aging in the general population. — SMH
Nature Med. 7, 235 (2005).