Results 31 to 40 of about 2,732 (185)

Progerin-expressing endothelial cells are unable to adapt to shear stress [PDF]

Biophysical Journal, 2021
Abstract Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare premature aging disease caused by a single-point mutation in the lamin A gene, resulting in a truncated and farnesylated form of lamin A. This mutant lamin A protein, known as progerin, accumulates at the periphery of the nuclear lamina, resulting in both an abnormal nuclear
Brooke E. Danielsson, Hannah C. Peters, Kranthi Bathula, Lindsay M. Spear, Natalie A. Noll, Kris N. Dahl, Daniel E. Conway   +6 more
openaire   +2 more sources

progerin overexpression in C3h10 cells RNAseq.xls [PDF]

, 2023
we overexpressed progerin in the C3H10 cell line and performed RNA sequencing analysis. 106 genes were down-regulated and 254 genes were up-regulated in progerin expressed cells with fold change greater than 1.5 and P-value less than 0.05, compared with ...
jinlong suo (15151015)
core   +1 more source

Identification of differential protein interactors of lamin A and progerin [PDF]

Nucleus, 2010
The nuclear lamina is an interconnected meshwork of intermediate filament proteins underlying the nuclear envelope. The lamina is an important regulator of nuclear structural integrity as well as nuclear processes, including transcription, DNA replication and chromatin remodeling. The major components of the lamina are A- and B-type lamins.
Kubben, N, Voncken, JW, Demmers, Jeroen, Calis, C, van Almen, G, Pinto, Y, Misteli, T   +6 more
openaire   +3 more sources

Prematurely Aged Human Microglia Exhibit Impaired Stress Response and Defective Nucleocytoplasmic Shuttling of ALS Associated FUS. [PDF]

Aging Cell
We developed a human microglia aging model by doxycycline‐induced GFP‐progerin expression, which offers a valuable tool to study human microglial aging and its contribution to neurodegeneration. Induced HMC3‐Progerin cells exhibited increased age markers as well as transcriptomic alterations, including an age‐associated transcriptomic shift and ...
Hartmann C, Haß C, Knobloch M, Barrantes I, Fumagalli L, Premereur J, Markert F, Peters M, Koromila G, Hartmann A, Jäger K, Abel J, Mancuso R, Storch A, Walter M, Fuellen G, Hermann A.   +16 more
europepmc   +2 more sources

miR-140-5p Overexpression Contributes to Oxidative Stress and Mitochondrial Dysfunction in Hutchinson-Gilford Progeria Syndrome Fibroblasts Through NRF2 Pathway. [PDF]

Aging Cell
This study identifies a novel molecular mechanism involving miR‐140‐5p that contributes to the pathogenesis of HGPS. By decreasing NRF2 expression, miR‐140‐5p overexpression results in downregulation of the NRF2/KEAP1/HO‐1 antioxidant pathway in HGPS fibroblasts, leading to increased oxidative stress and mitochondrial dysfunction, two hallmarks of ...
Toury L, Frankel D, Nael S, Abaji M, Le Goff L, Basset M, Airault C, Vernay B, Novoa-Del-Toro EM, Bartoli C, Baudot A, Magdinier F, Kaspi E, Roll P.   +13 more
europepmc   +2 more sources

Oxidative stress-induced premature senescence and aggravated denervated skeletal muscular atrophy by regulating progerin–p53 interaction

Skeletal Muscle, 2022
Background Progerin elevates atrophic gene expression and helps modify the nuclear membrane to cause severe muscle pathology, which is similar to muscle weakness in the elderly, to alter the development and function of the skeletal muscles.
Yaoxian Xiang, Zongqi You, Xinying Huang, Junxi Dai, Junpeng Zhang, Shuqi Nie, Lei Xu, Junjian Jiang, Jianguang Xu   +8 more
doaj   +1 more source

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, Adriaens, Michiel, Meuleman, Wouter, Voncken, Jan W, van Steensel, Bas, Misteli, Tom   +5 more
openaire   +2 more sources

Expression of progerin does not result in an increased mutation rate [PDF]

Chromosome Research, 2017
In the premature ageing disease Hutchinson-Gilford progeria syndrome (HGPS), the underlying genetic defect in the lamin A gene leads to accumulation at the nuclear lamina of progerin-a mutant form of lamin A that cannot be correctly processed. This has been reported to result in defects in the DNA damage response and in DNA repair, leading to the ...
Deniaud, Emmanuelle, Lemaitre, Charlene, Boyle, Shelagh, Bickmore, Wendy; id_orcid 0000-0001-6660-7735   +3 more
openaire   +3 more sources

Investigation of progerin expression in non-Hutchinson-Gilford Progeria Syndrome individuals [PDF]

, 2023
Hutchinson-Gilford Progerin Syndrome (HGPS) is a premature aging disease caused by a point mutation in the LMNA gene, which encodes A-type lamins. This mutation activates a cryptic splice donor in exon 11 and leads to the production of a toxic lamin ...
Yu, Reynold
core   +1 more source

ESCRT-III controls nuclear envelope deformation induced by progerin [PDF]

Scientific Reports, 2020
AbstractHutchinson-Gilford progeria syndrome (HGPS) is a premature aging disorder, caused by mutation in the gene encoding lamin A/C, which produces a truncated protein called progerin. In cells from HGPS patients, progerin accumulates at the nuclear membrane (NM), where it causes NM deformations. In this study, we investigated whether progerin-induced
Arii, Jun, Maeda, Fumio, Maruzuru, Yuhei, Koyanagi, Naoto, Kato, Akihisa, Mori, Yasuko, Kawaguchi, Yasushi   +6 more
openaire   +2 more sources