[HTML][HTML] Eliminating the synthesis of mature lamin A reduces disease phenotypes in mice carrying a Hutchinson-Gilford progeria syndrome allele
SH Yang, X Qiao, E Farber, SY Chang, LG Fong… - Journal of biological …, 2008 - ASBMB
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 heterozygous mice (Lmna HG/+) exhibit
many phenotypes of progeria. In this study, we tested the hypothesis that the phenotypes …
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 heterozygous mice (Lmna HG/+) exhibit
many phenotypes of progeria. In this study, we tested the hypothesis that the phenotypes …