Results 31 to 40 of about 7,722 (240)
Cells, 2023 Hutchinson-Gilford progeria syndrome (HGPS) is a rare premature aging disorder caused by the expression of progerin, a mutant variant of Lamin A. Recently, HGPS studies have gained relevance because unraveling its underlying mechanism would help to ...
Feliciano Monterrubio-Ledezma +6 more
doaj +1 more source
Life Science Alliance, 2021 The findings show that increased lysyl oxidase abundance is causal for the elevated arterial stiffness present in the arteries of Hutchinson–Gilford Progeria Syndrome mice.
Ryan von Kleeck +8 more
doaj +1 more source
Loss of H3K9me3 correlates with impaired ATM activation in HGPS. [PDF]
, 2016 (A). Representative fluorescence images of gammaH2AX and H3K9me3 in middle passage normal and HGPS fibroblasts after Dox treatment. “Low H3K9me3” is defined as any H3K9me3 fluorescence intensity that falls below the lower 5% of the normal population ...
Celeste Witting (3372776) +6 more
core +1 more source
Sim-Ptychography Imaging of Hutchinson-Gilford Progeria Syndrome (HGPS) Cells [PDF]
, 2021 180a Wednesday, February 24, 2021 ptychographic module was developed and mounted on a commercial SIM, to simultaneously collect ptychography and SIM data.
Cainero, Isotta +3 more
core +1 more source
HGPS fibroblasts show reduced gammaH2AX response upon Dox treatment. [PDF]
, 2016 (A) Representative fluorescence images of gammaH2AX foci in middle passage normal and HGPS fibroblasts after Dox treatment. Scale Bar: 5um. (B).
Celeste Witting (3372776) +6 more
core +1 more source
Cells, 2021 Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disease that recapitulates many symptoms of physiological aging and precipitates death. Patients develop severe vascular alterations, mainly massive vascular smooth muscle cell loss, vessel ...
Ignacio Benedicto +2 more
doaj +1 more source
Disrupting the LINC Complex in Smooth Muscle Cells Reduces Aortic Disease in a Mouse Model of HGPS
Science Translational Medicine, 2018 Disrupting the LINC complex reduces aortic disease in a mouse model of Hutchinson-Gilford progeria syndrome. Help for HGPS Children with Hutchinson-Gilford progeria syndrome (HGPS), a rare disorder of accelerated aging caused by mutations in lamin ...
Paul H. Kim +12 more
semanticscholar +1 more source
In Vivo Base Editing Rescues Hutchinson-Gilford Progeria Syndrome in Mice
Nature, 2021 Hutchinson–Gilford progeria syndrome (HGPS or progeria) is typically caused by a dominant-negative C•G-to-T•A mutation (c.1824 C>T; p.G608G) in LMNA, the gene that encodes nuclear lamin A.
Luke W. Koblan +20 more
semanticscholar +1 more source
Temsirolimus does not impact HGPS mitochondrial dysfunction. [PDF]
, 2016 (A) Immunohistochemistry was performed on mock-treated or temsirolimus-treated control (GMO3349C) and HGPS (HGADFN003) fibroblasts (9 days of treatment). Antibodies against NADPH oxidase subunit 4 (Nox4) and progerin were used.
Diana Gabriel (3608093) +2 more
core +1 more source
Methylene blue restores H3K9me3 and rescues DDR defects in HGPS. [PDF]
, 2016 (A). Western blotting analysis with anti-pATM(S1981), anti-ATM, anti- gammaH2AX, anti-HP1alpha, anti-H3K9me3 and anti-GAPDH antibodies on late passage normal and HGPS fibroblasts treated with or without MB after 30 days.
Celeste Witting (3372776) +6 more
core +1 more source