Results 1 to 10 of about 2,950 (183)
ZMPSTE24 Is Associated with Elevated Inflammation and Progerin mRNA [PDF]
Cells, 2020 Lamins are important filaments forming the inner nuclear membrane. Lamin A is processed by zinc metalloproteinase (ZMPSTE24). Failure to cleave a truncated form of prelamin A—also called progerin—causes Hutchinson–Gilford progeria syndrome a well-known ...
Marc-Michael Zaruba, Michael Graber
exaly +6 more sources
The syntaxin-binding protein STXBP5 regulates progerin expression
Scientific ReportsHutchinson–Gilfor progeria syndrome (HGPS) is caused by a mutation in Lamin A resulting in the production of a protein called progerin. The accumulation of progerin induces inflammation, cellular senescence and activation of the P53 pathway.
Xinyi Lu, Ningbo Yu
exaly +5 more sources
MG 132‐induced progerin clearance is mediated by autophagy activation and splicing regulation
EMBO Molecular Medicine, 2017 Hutchinson–Gilford progeria syndrome (HGPS) is a lethal premature and accelerated aging disease caused by a de novo point mutation in LMNA encoding A‐type lamins.
Nicolas Levy, Claire Navarro
exaly +6 more sources
First Generation Proteolysis Targeting Chimeras (PROTACs) for the Treatment of Progeria [PDF]
Advanced ScienceHutchinson‐Gilford progeria syndrome (HGPS) is a rare genetic disorder caused by a mutation in the LMNA gene, leading to the production of progerin, an aberrant and toxic form of lamin A. Due to its hydrophobic nature, progerin accumulates at the nuclear
Jon Macicior‐Michelena +5 more
doaj +3 more sources
Cells, 2020 Hutchinson–Gilford progeria syndrome (HGPS) is a rare premature aging disorder notably characterized by precocious and deadly atherosclerosis. Almost 90% of HGPS patients carry a LMNA p.G608G splice variant that leads to the expression of a permanently ...
Jacqueline Capeau +2 more
exaly +4 more sources
Progerin, an Aberrant Spliced Form of Lamin A, Is a Potential Therapeutic Target for HGPS
Cells, 2023 Hutchinson–Gilford progeria syndrome (HGPS) is an extremely rare genetic disorder caused by the mutant protein progerin, which is expressed by the abnormal splicing of the LMNA gene.
Bum-Joon Park +2 more
exaly +5 more sources
Cells, 2022 Progerin, a permanently farnesylated prelamin A protein in cell nuclei, is potentially implicated in the defenestration of liver sinusoidal endothelial cells (LSECs) and liver fibrogenesis.
Bingyong Zhang +2 more
exaly +3 more sources
The unfolded protein response in progeria arteries originates from non-endothelial cell types [PDF]
Life Science AllianceProgeroid endothelial cells do not show a robust activation of the unfolded protein response, contrary to what has been reported in other arterial cell types such as vascular smooth muscle cells. Hutchinson-Gilford Progeria Syndrome (HGPS) is a premature
Raquel A Silva +4 more
doaj +2 more sources
The accumulation of progerin underlies the loss of aortic smooth muscle cells in Hutchinson-Gilford progeria syndrome [PDF]
Cell Death and DiseaseHutchinson-Gilford progeria syndrome (HGPS) is caused by progerin, an internally truncated prelamin A that does not undergo the ZMPSTE24 processing step that releases prelamin A’s farnesylated carboxyl terminus; consequently, progerin remains ...
Paul H. Kim +9 more
doaj +2 more sources
Cells, 2020 Cardiovascular disease (CVD) is the main cause of death worldwide, and aging is its leading risk factor. Aging is much accelerated in Hutchinson−Gilford progeria syndrome (HGPS), an ultra-rare genetic disorder provoked by the ubiquitous expression ...
Vicente Andres +2 more
exaly +3 more sources