[HTML][HTML] Molecular insights into the premature aging disease progeria

S Vidak, R Foisner - Histochemistry and cell biology, 2016 - Springer
Hutchinson–Gilford progeria syndrome (HGPS) is an extremely rare premature aging
disease presenting many features resembling the normal aging process. HGPS patients die …

Hallmarks of progeroid syndromes: lessons from mice and reprogrammed cells

D Carrero, C Soria-Valles… - Disease models & …, 2016 - journals.biologists.com
Ageing is a process that inevitably affects most living organisms and involves the
accumulation of macromolecular damage, genomic instability and loss of heterochromatin …

Clinical trial of the protein farnesylation inhibitors lonafarnib, pravastatin, and zoledronic acid in children with Hutchinson-Gilford progeria syndrome

LB Gordon, ME Kleinman, J Massaro… - Circulation, 2016 - Am Heart Assoc
Background: Hutchinson-Gilford progeria syndrome is an extremely rare, fatal, segmental
premature aging syndrome caused by a mutation in LMNA yielding the farnesylated …

[HTML][HTML] Modulation of LMNA splicing as a strategy to treat prelamin A diseases

JM Lee, C Nobumori, Y Tu, C Choi… - The Journal of …, 2016 - Am Soc Clin Investig
The alternatively spliced products of LMNA, lamin C and prelamin A (the precursor to lamin
A), are produced in similar amounts in most tissues and have largely redundant functions …

[HTML][HTML] Vascular aging: implications for cardiovascular disease and therapy

YT Ghebre, E Yakubov, WT Wong… - Translational …, 2016 - ncbi.nlm.nih.gov
The incidence and prevalence of cardiovascular disease is highest among the elderly, in
part, due to deleterious effects of advancing age on the heart and blood vessels. Aging, a …

[HTML][HTML] Interruption of progerin–lamin A/C binding ameliorates Hutchinson-Gilford progeria syndrome phenotype

SJ Lee, YS Jung, MH Yoon, S Kang… - The Journal of …, 2016 - Am Soc Clin Investig
Hutchinson-Gilford progeria syndrome (HGPS) is a rare autosomal dominant genetic
disease that is caused by a silent mutation of the LMNA gene encoding lamins A and C …

[HTML][HTML] Vitamin D receptor signaling improves Hutchinson-Gilford progeria syndrome cellular phenotypes

R Kreienkamp, M Croke, MA Neumann, G Bedia-Diaz… - Oncotarget, 2016 - ncbi.nlm.nih.gov
Abstract Hutchinson-Gilford Progeria Syndrome (HGPS) is a devastating incurable
premature aging disease caused by accumulation of progerin, a toxic lamin A mutant …

A mutation abolishing the ZMPSTE24 cleavage site in prelamin A causes a progeroid disorder

Y Wang, U Lichter-Konecki… - Journal of cell …, 2016 - journals.biologists.com
In 1994 in the Journal of Cell Science, Hennekes and Nigg reported that changing valine to
arginine at the endoproteolytic cleavage site in chicken prelamin A abolishes its conversion …

[HTML][HTML] Metformin decreases progerin expression and alleviates pathological defects of Hutchinson–Gilford progeria syndrome cells

AL Egesipe, S Blondel, A Lo Cicero… - NPJ aging and …, 2016 - nature.com
Hutchinson–Gilford progeria syndrome (HGPS) is a rare genetic disorder that causes
systemic accelerated aging in children. This syndrome is due to a mutation in the LMNA …

[HTML][HTML] Cell mechanosensitivity is enabled by the LINC nuclear complex

G Uzer, CT Rubin, J Rubin - Current molecular biology reports, 2016 - Springer
Mechanoresponses in mesenchymal stem cells (MSCs) guide both differentiation and
function. In this review, we focus on advances in our understanding of how the cytoplasmic …