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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Human WRN is an intrinsic inhibitor of progerin, abnormal splicing product of lamin A [PDF]
Scientific Reports, 2021 Werner syndrome (WRN) is a rare progressive genetic disorder, caused by functional defects in WRN protein and RecQ4L DNA helicase. Acceleration of the aging process is initiated at puberty and the expected life span is approximately the late 50 s ...
So-mi Kang +11 more
doaj +3 more sources
Role of progerin-induced telomere dysfunction in HGPS premature cellular senescence [PDF]
Journal of Cell Science, 2010 Hutchinson-Gilford Progeria Syndrome (HGPS) is a premature-aging syndrome caused by a dominant mutation in the gene encoding lamin A, which leads to an aberrantly spliced and processed protein termed progerin. Previous studies have shown that progerin induces early senescence associated with increased DNA-damage signaling and that telomerase extends ...
Erica K, Benson +2 more
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New insights in Progerin-induced modifications of chromatin landscapes
Biophysical Journal, 2023 Genome structure, expression, and regulation are crucial in maintaining the physiological state of any cell. However, even a single variation in one of these processes can induce genomic instability, leading to different pathologies, including aging and cancer.
Cainero, I +5 more
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MG132 Induces Progerin Clearance and Improves Disease Phenotypes in HGPS-like Patients’ Cells
Cells, 2022 Progeroid syndromes (PS), including Hutchinson-Gilford Progeria Syndrome (HGPS), are premature and accelerated aging diseases, characterized by clinical features mimicking physiological aging.
Nicolas Levy, Koffi Mawuse Guedenon
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Rare progerin-expressing preadipocytes and adipocytes contribute to tissue depletion over time
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
doaj +2 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
doaj +2 more sources
Progerin Hinders Autophagy Flux at Its Final Stages in Hutchinson-Gilford Progeria Syndrome Cells, Preventing Its Own Autophagic Degradation. [PDF]
Aging CellWe demonstrated that progerin induces the formation of autophagosomes but impairs their maturation and subsequent fusion with lysosomes. Progerin impedes proper autophagy flux, thus preventing its own autophagic degradation. Selinexor treatment improved both autophagosome maturation and autophagosome‐lysosome fusion, which ultimately led to effective ...
García-Aguirre I +18 more
europepmc +2 more sources
DEVELOPMENT OF AN ACCELERATED ALZHEIMER’S DISEASE IN VITRO MODEL WITH THE ADDITION OF PROGERIN
, 2023 Alzheimer’s Disease (AD) is one of the most common causes of dementia. Twopathological features of AD include amyloid plaques and neurofibrillary tangles. The mechanism underlying the disease's onset and progression remains unclear. Lamin A is an essential component of the nuclear lamina, and nuclear lamina plays a vital role in essential cell ...
Xue, Huijing
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Mapping of lamin A- and progerin-interacting genome regions [PDF]
Chromosoma, 2012 Mutations in the A-type lamins A and C, two major components of the nuclear lamina, cause a large group of phenotypically diverse diseases collectively referred to as laminopathies. These conditions often involve defects in chromatin organization.
Kubben, Nard +5 more
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