Lamins are cool!!!

Lamin A-Dependent Nuclear Defects in Human Aging

Paola Scaffidi ¹ and Tom Misteli ^1*
Published online April 27 2006; 10.1126/science.1127168 (Science Express Reports)

Lamins are intermediate filament proteins and are the main component of the nuclear lamina. Although these proteins are expressed in different tissues, mutations in the LMN gene that encodes Lamin A and Lamin C, are associated with distinct genetic disorders such as muscular dystrophies, lypodystrophies and progerias.

Now it was found that the same mutation on lamin A protein associated with progeria, a rare disease were children suffer from premature aging, also accumulates in elderly human cells. This mutation originates a truncated version of lamin A.

Two key findings are reported:
- Traces of this truncated version are also observed in cells from young humans, although the protein does not accumulate, as observed in elderly human cells. This suggests that younger cells have mechanisms to destroy this truncated version.
- Cell nuclei from elderly humans have the same morphology and DNA-damage levels as cell nuclei from progeria patients. Impressively, this morphology is reversed when the production of the truncated protein is prevented, by expression of a morpholino targeting the "truncated mRNA".

Maybe in the future cell aging can be prevented by inhibiting the formation of truncated lamin A.... It will be cool to generate a trangenic mice expressing the morpholino that inhibits the formation of spontaneous truncated lamin A.