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
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The syntaxin-binding protein STXBP5 regulates progerin expression [PDF]
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, Weiyu Zhang, Lu Xinyi
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MG 132‐induced progerin clearance is mediated by autophagy activation and splicing regulation [PDF]
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
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Progerin-Induced Impairment in Wound Healing and Proliferation in Vascular Endothelial Cells [PDF]
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 ...
Julie Y Ji
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Promotion of tumor development in prostate cancer by progerin [PDF]
Cancer Cell International, 2010 Progerin is a truncated form of lamin A. It is identified in patients with Hutchinson-Gilford progeria syndrome (HGPS), a disease characterized by accelerated aging.
Nie Daotai +3 more
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Antisense oligonucleotide induction of progerin in human myogenic cells. [PDF]
PLoS ONE, 2014 We sought to use splice-switching antisense oligonucleotides to produce a model of accelerated ageing by enhancing expression of progerin, translated from a mis-spliced lamin A gene (LMNA) transcript in human myogenic cells. The progerin transcript (LMNA
Yue-Bei Luo +6 more
doaj +8 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
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Upregulation of the aging related LMNA splice variant progerin in dilated cardiomyopathy. [PDF]
PLoS ONE, 2018 Mutations in the LMNA gene are a common cause (6-8%) of dilated cardiomyopathy (DCM) leading to heart failure, a growing health care problem worldwide. The premature aging disease Hutchinson-Gilford syndrome (HGPS) is also caused by defined mutations in ...
Moritz Messner +6 more
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New look at the role of progerin in skin aging [PDF]
Menopause Review, 2015 Current literature data indicate that progerin, which is a mutant of lamin A, may be one of several previously known physiological biomarkers of the aging process which begins at the age of 30. Lamins belong to the family of intermediate filaments type V
Anna Skoczyńska +3 more
doaj +5 more sources
Progerin Expression Induces Inflammation, Oxidative Stress and Senescence in Human Coronary Endothelial Cells [PDF]
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
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