Results 21 to 30 of about 3,816 (187)

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

Baricitinib Augments Lonafarnib Therapy to Preserve Colonic Homeostasis and Microbial Balance in a Mouse Model of Progeria. [PDF]

Aging Cell
Combined baricitinib and lonafarnib treatment improved colonic pathology in LmnaG609G/G609G progeria mice by reducing progerin, maintaining epithelial regeneration, mitigating inflammation and senescence, and preserving microbiome composition. ABSTRACT Hutchinson‐Gilford Progeria Syndrome (HGPS) is a fatal genetic disorder caused by progerin, a mutant ...
Schroll M, Amar Y, Krüger P, Neuhaus K, Djabali K.   +4 more
europepmc   +2 more sources

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

Cardiomyocyte-Restricted Expression of Progerin Confers Cardiac Hypertrophy. [PDF]

Circ Res
Subati T, Buehler DP, Zhong L, Miller FJ, Agrawal V, Brown JD.   +5 more
europepmc   +2 more sources

A new fluorescent probe for the visualization of progerin

Bioorganic Chemistry, 2023
Hutchinson-Gilford progeria syndrome (HGPS) or progeria is a rare genetic disease that causes premature aging, leading to a drastic reduction in the life expectancy of patients. Progeria is mainly caused by the intracellular accumulation of a defective protein called progerin, generated from a mutation in the LMNA gene.
Macicior Michelena, Jon, Fernández, Daniel, Ortega Gutiérrez, Silvia   +2 more
openaire   +4 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

Generation of Nonintegrative-Induced Pluripotent Stem Cells in Hutchinson-Gilford Progeria Syndrome: Enhancing Aging Research. [PDF]

Aging Med (Milton)
We generated genetically stable iPSCs from HGPS patients using a nonintegrative Sendai virus method. This approach preserves cellular integrity, enhances disease modeling, and enables accurate study of Progerin‐induced cardiovascular dysfunction. Overall, it provides a reliable platform for understanding HGPS and aging‐related disease pathology and ...
Kadiwala J, Shakur R.
europepmc   +2 more sources

Nucleophagic Degradation of Progerin Ameliorates Defenestration in Liver Sinusoidal Endothelium Due to SIRT1-Mediated Deacetylation of Nuclear LC3

Cells, 2022
Progerin, a permanently farnesylated prelamin A protein in cell nuclei, is potentially implicated in the defenestration of liver sinusoidal endothelial cells (LSECs) and liver fibrogenesis.
Yangqiu Bai, Jinying Liu, Xiaoke Jiang, Xiuling Li, Bingyong Zhang, Xiaoying Luo   +5 more
doaj   +1 more source

Longwave UV Light Induces the Aging-Associated Progerin [PDF]

Journal of Investigative Dermatology, 2013
Premature aging in Hutchinson-Gilford progeria syndrome (HGPS) is caused by a mutation of the LMNA gene that activates a cryptic splice site. This results in expression of a truncated form of Lamin A, called progerin. Accumulation of progerin in the nuclei of HGPS cells impairs nuclear functions and causes abnormal nuclear morphology.
Takeuchi, Hirotaka, Rünger, Thomas M.
openaire   +2 more sources

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.
Harhouri, Karim, Navarro, Claire, Baquerre, Camille, da Silva, Nathalie, Bartoli, Catherine, Casey, Frank, Mawuse, Guedenon, Doubaj, Yassamine, Lévy, Nicolas, de Sandre-Giovannoli, Annachiara   +9 more
openaire   +3 more sources