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.
Hongqian Qi +5 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 +3 more sources
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
doaj +3 more sources
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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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
doaj +4 more sources
Nuclear envelope budding inhibition slows down progerin-induced aging process [PDF]
Proceedings of the National Academy of Sciences of the United States of AmericaProgerin causes Hutchinson–Gilford progeria syndrome (HGPS), but how progerin accelerates aging is still an interesting question. Here, we provide evidence linking nuclear envelope (NE) budding and accelerated aging. Mechanistically, progerin disrupts nuclear lamina to induce NE budding in concert with lamin A/C, resulting in transport of chromatin ...
, He Ren, Qiaoyu Lin
exaly +4 more sources
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
doaj +3 more sources
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
doaj +4 more sources
Progerin can induce DNA damage in the absence of global changes in replication or cell proliferation. [PDF]
PLoS ONEHutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic condition characterized by features of accelerated aging, and individuals with HGPS seldom live beyond their mid-teens.
Liza A Joudeh +5 more
doaj +2 more sources