Results 21 to 30 of about 2,732 (185)

Progerin reduces LAP2α-telomere association in Hutchinson-Gilford progeria

open access: yeseLife, 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
doaj   +6 more sources

Human WRN is an intrinsic inhibitor of progerin, abnormal splicing product of lamin A [PDF]

open access: yesScientific 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]

open access: yesJournal 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
openaire   +4 more sources

New insights in Progerin-induced modifications of chromatin landscapes [PDF]

open access: yesBiophysical 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
openaire   +2 more sources

Selection of specific and efficient siRNAs in new cellular model for Hutchinson-Gilford progeria syndrome therapy [PDF]

open access: yesMolecular Therapy: Nucleic Acids
Hutchinson-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

Transcriptional profiling of Hutchinson-Gilford progeria patients identifies primary target pathways of progerin [PDF]

open access: yesNucleus
Hutchinson Gilford Progeria Syndrome (HGPS) is an ultra-rare pediatric premature aging disorder. It is caused by a point mutation in the LMNA gene leading to the production of the dominant-negative progerin isoform of the nuclear envelope protein lamin A.
Sandra Vidak, Sohyoung Kim, Tom Misteli
doaj   +2 more sources

Progerin mRNA Is Associated with Smoking and Signs of Increased Microvascular Damage in Patients with Diabetic Macular Edema [PDF]

open access: yesInternational Journal of Molecular Sciences
The premature aging disease Hutchinson–Gilford Syndrome (HGPS) is caused by defined mutations in the LMNA gene, resulting in the activation of a cryptic splice donor site, which leads to a defective truncated prelamin A protein called progerin ...
Vivek Jeyakumar   +2 more
exaly   +4 more sources

Rare progerin-expressing preadipocytes and adipocytes contribute to tissue depletion over time [PDF]

open access: yesScientific 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

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