Results 1 to 10 of about 3,871 (195)
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
exaly +5 more sources
Progerin-Induced Impairment in Wound Healing and Proliferation in Vascular Endothelial Cells
Frontiers in Aging, 2022 Progerin as a mutated isoform of lamin A protein was first known to induce premature atherosclerosis progression in patients with Hutchinson-Gilford progeria syndrome (HGPS), and its role in provoking an inflammatory response in vascular cells and ...
Yizhi Jiang
exaly +5 more sources
Attenuated Nuclear Tension Regulates Progerin‐Induced Mechanosensitive Nuclear Wrinkling and Chromatin Remodeling [PDF]
Advanced ScienceHutchinson–Gilford progeria syndrome, caused by a mutation in the LMNA gene, leads to increased levels of truncated prelamin A, progerin, in the nuclear membrane. The accumulation of progerin results in defective nuclear morphology and is associated with
Ji‐Eun Park +9 more
doaj +5 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 ...
Guillaume Bidault +2 more
exaly +3 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
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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
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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.
yangqiu bai, LIU JYING, Xiuling Li
exaly +3 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 ...
Lara del Campo Milán +2 more
exaly +3 more sources
Selection of specific and efficient siRNAs in new cellular model for Hutchinson-Gilford progeria syndrome therapy [PDF]
Molecular Therapy: Nucleic AcidsHutchinson-Gilford progeria syndrome is a fatal genetic disorder caused by a point mutation in the gene encoding the nuclear envelope protein lamin A/C.
Volha Dzianisava +3 more
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Transcriptional profiling of Hutchinson-Gilford progeria patients identifies primary target pathways of progerin [PDF]
NucleusHutchinson Gilford Progeria Syndrome (HGPS) is an ultra-rare pediatric premature aging disorder. It is caused by a point mutation in the LMNA gene leading to the production of the dominant-negative progerin isoform of the nuclear envelope protein lamin A.
Sandra Vidak, Sohyoung Kim, Tom Misteli
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