[HTML][HTML] Molecular insights into the premature aging disease progeria
Hutchinson–Gilford progeria syndrome (HGPS) is an extremely rare premature aging
disease presenting many features resembling the normal aging process. HGPS patients die …
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 …
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 …
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 …
A), are produced in similar amounts in most tissues and have largely redundant functions …
[HTML][HTML] Vascular aging: implications for cardiovascular disease and therapy
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 …
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 …
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 …
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 …
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 …
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
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 …
function. In this review, we focus on advances in our understanding of how the cytoplasmic …