Article
Eliminating the synthesis of mature lamin A reduces disease phenotypes in mice carrying a Hutchinson-Gilford progeria syndrome allele.
The Journal of biological chemistry - 14 Mar 2008
Yang Shao H, Qiao Xin, Farber Emily, Chang Sandy Y, Fong Loren G, Young Stephen G
Abstract excerpt
Hutchinson-Gilford progeria syndrome is caused by the synthesis of a mutant form of prelamin A, which is generally called progerin. Progerin is targeted to the nuclear rim, where it interferes with the integrity of the nuclear lamina, causes misshapen cell nuclei, and leads to multiple aging-like disease phenotypes. We created a gene-targeted allele yielding exclusively progerin (Lmna HG) and found that...
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