Impact of miR-181a on SIRT1 Expression and Senescence in Hutchinson-Gilford Progeria Syndrome. [PDF]
Lederer EM +5 more
europepmc +1 more source
The anti-senescence effect of D-β-hydroxybutyrate in Hutchinson-Gilford progeria syndrome involves progerin clearance by the activation of the AMPK-mTOR-autophagy pathway. [PDF]
Monterrubio-Ledezma F +5 more
europepmc +1 more source
Analysis of Beta-Dystroglycan in Different Cell Models of Senescence. [PDF]
Jimenez-Gutierrez GE +8 more
europepmc +1 more source
Disease pathogenicity in Hutchinson-Gilford progeria syndrome mice: insights from lung-associated alterations. [PDF]
Wang J +14 more
europepmc +1 more source
Old enough to be a model? On the role of maturity in stem cell-based models for neuropsychiatric disorders. [PDF]
He B, Smedler E.
europepmc +1 more source
Single-cell RNA sequencing analysis reveals the critical role of fibroblasts in aortic progeria-associated vascular remodeling in Hutchinson-Gilford progeria syndrome mice. [PDF]
Sun Q +6 more
europepmc +1 more source
High Body Mass Index is Associated with Elevated Blood Levels of Progerin mRNA [PDF]
Obesity is a well-described risk factor resulting in premature aging of the cardiovascular system ultimately limiting longevity. Premature cardiac death and aging is the hallmark of Hutchinson–Gilford syndrome (HGPS), a disease caused by defined ...
Marc-Michael Zaruba
exaly +2 more sources
Antisense-Based Progerin Downregulation in HGPS-Like Patients’ Cells [PDF]
Progeroid laminopathies, including Hutchinson-Gilford Progeria Syndrome (HGPS, OMIM #176670), are premature and accelerated aging diseases caused by defects in nuclear A-type Lamins. Most HGPS patients carry a de novo point mutation within exon 11 of the LMNA gene encoding A-type Lamins.
Nicolas Levy, Claire Navarro
exaly +4 more sources
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Progerin and Its Role in Accelerated and Natural Aging
Molecular Biology, 2022Well-known theories of aging suggest that a certain metabolic defect negatively affects vital activity of the cell, be it oxidative stress, the accumulation of lesions in DNA, the exhaustion of telomeres, or distorted epigenetic processes. The theory of aging considered in the review postulates that an accumulation of progerin on the inner side of the ...
openaire +2 more sources
Corruption of DNA end-joining in mammalian chromosomes by progerin expression
DNA Repair, 2023Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic condition characterized by features of accelerated aging and a life expectancy of about 14 years. HGPS is commonly caused by a point mutation in the LMNA gene which codes for lamin A, an essential component of the nuclear lamina.
Liza A, Joudeh +9 more
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