Author Correction: The syntaxin-binding protein STXBP5 regulates progerin expression. [PDF]
Sci RepQi H, Wu Y, Zhang W, Yu N, Lu X, Liu J.
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BMI1 represses G-quadruplex DNA formation to maintain genomic stability during replication. [PDF]
J Biol ChemHanna R, Deneault E, Bernier G.
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General anesthesia in patient with Hutchinson-Gilford Progeria syndrome: two case reports of dental treatment in the one patient. [PDF]
BMC AnesthesiolMankovsky B +3 more
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Vascular Smooth Muscle–Specific Progerin Expression Accelerates Atherosclerosis and Death in a Mouse Model of Hutchinson-Gilford Progeria Syndrome [PDF]
Circulation, 2018 Background: Progerin, an aberrant protein that accumulates with age, causes the rare genetic disease Hutchinson-Gilford progeria syndrome (HGPS). Patients who have HGPS exhibit ubiquitous progerin expression, accelerated aging and atherosclerosis, and ...
Magda R Hamczyk +2 more
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Antisense-Based Progerin Downregulation in HGPS-Like Patients’ Cells [PDF]
Cells, 2016 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.
Karim Harhouri +2 more
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New insights in Progerin-induced modifications of chromatin landscapes
Biophysical Journal, 2023Genome 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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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 ...
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Human iPSC-Based Modeling of Late-Onset Disease via Progerin-Induced Aging [PDF]
Cell Stem Cell, 2013 Reprogramming somatic cells to induced pluripotent stem cells (iPSCs) resets their identity back to an embryonic age and, thus, presents a significant hurdle for modeling late-onset disorders. In this study, we describe a strategy for inducing aging-related features in human iPSC-derived lineages and apply it to the modeling of Parkinson's disease (PD).
Jae-Won Shim +2 more
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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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