Publications
Research in plain language.
Selected papers on how the nuclear lamina is built, how progerin breaks it, and why that kills the smooth muscle cells of the aorta in progeria. Full list on Google Scholar.
Lamin B1 coordinates the assembly of the nuclear lamina
Rebuilt the nuclear lamina one lamin at a time in smooth muscle cells lacking all three lamin genes, showing that lamin B1 alone forms a normal meshwork and is needed for the other lamins to organize.
The accumulation of progerin underlies the loss of aortic smooth muscle cells in Hutchinson-Gilford progeria syndrome
Connected progressive progerin accumulation in the aorta to the selective loss of the smooth muscle cells that protect arterial structure.
Progerin forms an abnormal meshwork and has a dominant-negative effect on the nuclear lamina
Revealed large, irregular openings in the progerin meshwork and showed that higher lamin B1 expression can normalize its structure and reduce nuclear rupture.
Nuclear membrane ruptures underlie the vascular pathology in a mouse model of Hutchinson-Gilford progeria syndrome
Linked repetitive nuclear membrane rupture, mechanical stress, DNA damage, and smooth-muscle-cell death in progeria.
Disrupting the LINC complex in smooth muscle cells reduces aortic disease in a mouse model of Hutchinson-Gilford progeria syndrome
Showed that reducing force transmission to the nucleus protects smooth muscle cells and ameliorates arterial disease.