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
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Lamin microaggregates lead to altered mechanotransmission in progerin-expressing cells [PDF]
Nucleus, 2020 The nuclear lamina is a meshwork of intermediate filament proteins, and lamin A is the primary mechanical protein. An altered splicing of lamin A, known as progerin, causes the disease Hutchinson-Gilford progeria syndrome.
Brooke E. Danielsson +7 more
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Progerin reduces LAP2α-telomere association in Hutchinson-Gilford progeria
eLife, 2015 Hutchinson-Gilford progeria (HGPS) is a premature ageing syndrome caused by a mutation in LMNA, resulting in a truncated form of lamin A called progerin.
Alexandre Chojnowski +15 more
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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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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 ...
Moritz Messner +7 more
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Progerin mislocalizes myocardin-related transcription factor in Hutchinson–Guilford Progeria syndrome [PDF]
Vascular Biology, 2022 Hutchinson–Guilford Progeria syndrome (HGPS) is a rare genetic disease of premature aging and early death due to cardiovascular disease. The arteries of HGPS children and mice are pathologically stiff, and HGPS mice also display reduced arterial ...
Ryan von Kleeck +2 more
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MG132‐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.
Karim Harhouri +7 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 +3 more sources
Rare progerin-expressing preadipocytes and adipocytes contribute to tissue depletion over time [PDF]
Scientific Reports, 2017 Accumulation of progerin is believed to underlie the pathophysiology of Hutchinson-Gilford progeria syndrome, a disease characterized by clinical features suggestive of premature aging, including loss of subcutaneous white adipose tissue (sWAT). Although
Gwladys Revêchon +9 more
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miR-140-5p Overexpression Contributes to Oxidative Stress and Mitochondrial Dysfunction in Hutchinson-Gilford Progeria Syndrome Fibroblasts Through NRF2 Pathway. [PDF]
Aging CellThis study identifies a novel molecular mechanism involving miR‐140‐5p that contributes to the pathogenesis of HGPS. By decreasing NRF2 expression, miR‐140‐5p overexpression results in downregulation of the NRF2/KEAP1/HO‐1 antioxidant pathway in HGPS fibroblasts, leading to increased oxidative stress and mitochondrial dysfunction, two hallmarks of ...
Toury L +13 more
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