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https://www.readbyqxmd.com/read/30216637/targeting-the-phospholipase-a2-receptor-ameliorates-premature-aging-phenotypes
#1
Audrey Griveau, Clotilde Wiel, Benjamin Le Calvé, Dorian V Ziegler, Sophia Djebali, Marine Warnier, Nadine Martin, Jacqueline Marvel, David Vindrieux, Martin O Bergo, David Bernard
Hutchinson-Gilford progeria syndrome (HGPS) is a lethal premature aging that recapitulates many normal aging characteristics. This disorder is caused by mutation in the LMNA gene leading to the production of progerin which induces misshapen nuclei, cellular senescence, and aging. We previously showed that the phospholipase A2 receptor (PLA2R1) promotes senescence induced by replicative, oxidative, and oncogenic stress but its role during progerin-induced senescence and in progeria is currently unknown. Here, we show that knockdown of PLA2R1 prevented senescence induced by progerin expression in human fibroblasts and markedly delayed senescence of HGPS patient-derived fibroblasts...
September 14, 2018: Aging Cell
https://www.readbyqxmd.com/read/30157432/methionine-restriction-extends-lifespan-in-progeroid-mice-and-alters-lipid-and-bile-acid-metabolism
#2
Clea Bárcena, Pedro M Quirós, Sylvère Durand, Pablo Mayoral, Francisco Rodríguez, Xurde M Caravia, Guillermo Mariño, Cecilia Garabaya, María Teresa Fernández-García, Guido Kroemer, José M P Freije, Carlos López-Otín
Dietary intervention constitutes a feasible approach for modulating metabolism and improving the health span and lifespan. Methionine restriction (MR) delays the appearance of age-related diseases and increases longevity in normal mice. However, the effect of MR on premature aging remains to be elucidated. Here, we describe that MR extends lifespan in two different mouse models of Hutchinson-Gilford progeria syndrome (HGPS) by reversing the transcriptome alterations in inflammation and DNA-damage response genes present in this condition...
August 28, 2018: Cell Reports
https://www.readbyqxmd.com/read/30131443/extreme-nuclear-branching-in-healthy-epidermal-cells-of-the-xenopus-tail-fin
#3
Hannah E Arbach, Marcus Harland-Dunaway, Jessica K Chang, Andrea E Wills
Changes in nuclear morphology contribute to the regulation of complex cell properties, including differentiation and tissue elasticity. Perturbations of nuclear morphology are associated with pathologies that include progeria, cancer and muscular dystrophy. The mechanisms governing nuclear shape changes in healthy cells remain poorly understood, partially because there are few models of nuclear shape variation in healthy cells. Here, we introduce nuclear branching in epidermal fin cells of Xenopus tropicalis as a model for extreme variation of nuclear morphology in a diverse population of healthy cells...
September 20, 2018: Journal of Cell Science
https://www.readbyqxmd.com/read/30130743/epigenetic-clocks-galore-a-new-improved-clock-predicts-age-acceleration-in-hutchinson-gilford-progeria-syndrome-patients
#4
Andrew E Teschendorff
No abstract text is available yet for this article.
August 21, 2018: Aging
https://www.readbyqxmd.com/read/30127165/management-of-cataract-in-werner-syndrome
#5
Rakhi Kusumesh, Bibhuti Prassan Sinha, Anita Ambastha, Santosh Kumar Thakur
Werner syndrome (WS) is a rare progressive disorder. It is characterized by the appearance of unusually accelerated aging (progeria) including bilateral senile cataract. Here, we report a successful management of hypermature cataract in WS.
September 2018: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/30116163/assessment-of-the-aging-of-the-brown-adipose-tissue-by-1-8-f-fdg-pet-ct-imaging-in-the-progeria-mouse-model-lmna
#6
Zhengjie Wang, Xiaolong Xu, Yi Liu, Yongheng Gao, Fei Kang, Baohua Liu, Jing Wang
Brown adipose tissue (BAT) is an important energy metabolic organ that is highly implicated in obesity, type 2 diabetes, and atherosclerosis. Aging is one of the most important determinants of BAT activity. In this study, we used 18 F-FDG PET/CT imaging to assess BAT aging in Lmna-/- mice. The maximum standardized uptake value (SUVMax ) of the BAT was measured, and the target/nontarget (T/NT) values of BAT were calculated. The transcription and the protein expression levels of the uncoupling protein 1 (UCP1), beta3-adrenergic receptor ( β 3-AR), and the PR domain-containing 16 (PRDM16) were measured by quantitative real-time polymerase chain reaction (RT-PCR) and Western blotting or immunohistochemical analysis...
2018: Contrast Media & Molecular Imaging
https://www.readbyqxmd.com/read/30109767/altered-modulation-of-lamin-a-c-hdac2-interaction-and-p21-expression-during-oxidative-stress-response-in-hgps
#7
Elisabetta Mattioli, Davide Andrenacci, Cecilia Garofalo, Sabino Prencipe, Katia Scotlandi, Daniel Remondini, Davide Gentilini, Anna Maria Di Blasio, Sergio Valente, Emanuela Scarano, Lucia Cicchilitti, Giulia Piaggio, Antonello Mai, Giovanna Lattanzi
Defects in stress response are main determinants of cellular senescence and organism aging. In fibroblasts from patients affected by Hutchinson-Gilford progeria, a severe LMNA-linked syndrome associated with bone resorption, cardiovascular disorders, and premature aging, we found altered modulation of CDKN1A, encoding p21, upon oxidative stress induction, and accumulation of senescence markers during stress recovery. In this context, we unraveled a dynamic interaction of lamin A/C with HDAC2, an histone deacetylase that regulates CDKN1A expression...
August 15, 2018: Aging Cell
https://www.readbyqxmd.com/read/30069858/chemical-screen-identifies-a-geroprotective-role-of-quercetin-in-premature-aging
#8
Lingling Geng, Zunpeng Liu, Weiqi Zhang, Wei Li, Zeming Wu, Wei Wang, Ruotong Ren, Yao Su, Peichang Wang, Liang Sun, Zhenyu Ju, Piu Chan, Moshi Song, Jing Qu, Guang-Hui Liu
Aging increases the risk of various diseases. The main goal of aging research is to find therapies that attenuate aging and alleviate aging-related diseases. In this study, we screened a natural product library for geroprotective compounds using Werner syndrome (WS) human mesenchymal stem cells (hMSCs), a premature aging model that we recently established. Ten candidate compounds were identified and quercetin was investigated in detail due to its leading effects. Mechanistic studies revealed that quercetin alleviated senescence via the enhancement of cell proliferation and restoration of heterochromatin architecture in WS hMSCs...
August 1, 2018: Protein & Cell
https://www.readbyqxmd.com/read/30051577/increased-transport-of-acetyl-coa-into-the-endoplasmic-reticulum-causes-a-progeria-like-phenotype
#9
Yajing Peng, Samantha L Shapiro, Varuna C Banduseela, Inca A Dieterich, Kyle J Hewitt, Emery H Bresnick, Guangyao Kong, Jing Zhang, Kathryn L Schueler, Mark P Keller, Alan D Attie, Timothy A Hacker, Ruth Sullivan, Elle Kielar-Grevstad, Sebastian I Arriola Apelo, Dudley W Lamming, Rozalyn M Anderson, Luigi Puglielli
The membrane transporter AT-1/SLC33A1 translocates cytosolic acetyl-CoA into the lumen of the endoplasmic reticulum (ER), participating in quality control mechanisms within the secretory pathway. Mutations and duplication events in AT-1/SLC33A1 are highly pleiotropic and have been linked to diseases such as spastic paraplegia, developmental delay, autism spectrum disorder, intellectual disability, propensity to seizures, and dysmorphism. Despite these known associations, the biology of this key transporter is only beginning to be uncovered...
July 27, 2018: Aging Cell
https://www.readbyqxmd.com/read/30048243/epigenetic-clock-for-skin-and-blood-cells-applied-to-hutchinson-gilford-progeria-syndrome-and-ex-vivo-studies
#10
Steve Horvath, Junko Oshima, George M Martin, Ake T Lu, Austin Quach, Howard Cohen, Sarah Felton, Mieko Matsuyama, Donna Lowe, Sylwia Kabacik, James G Wilson, Alex P Reiner, Anna Maierhofer, Julia Flunkert, Abraham Aviv, Lifang Hou, Andrea A Baccarelli, Yun Li, James D Stewart, Eric A Whitsel, Luigi Ferrucci, Shigemi Matsuyama, Kenneth Raj
DNA methylation (DNAm)-based biomarkers of aging have been developed for many tissues and organs. However, these biomarkers have sub-optimal accuracy in fibroblasts and other cell types used in ex vivo studies. To address this challenge, we developed a novel and highly robust DNAm age estimator (based on 391 CpGs) for human fibroblasts, keratinocytes, buccal cells, endothelial cells, lymphoblastoid cells, skin, blood, and saliva samples. High age correlations can also be observed in sorted neurons, glia, brain, liver, and even bone samples...
July 26, 2018: Aging
https://www.readbyqxmd.com/read/30046839/electrocardiographic-abnormalities-in-patients-with-hutchinson-gilford-progeria-syndrome-reply
#11
Ashwin Prakash, Leslie Smoot
No abstract text is available yet for this article.
July 25, 2018: JAMA Cardiology
https://www.readbyqxmd.com/read/30046820/electrocardiographic-abnormalities-in-patients-with-hutchinson-gilford-progeria-syndrome
#12
David Filgueiras-Rama, José Rivera Torres, Vicente Andrés
No abstract text is available yet for this article.
July 25, 2018: JAMA Cardiology
https://www.readbyqxmd.com/read/30037605/cellular-stress-and-ampk-activation-as-a-common-mechanism-of-action-linking-the-effects-of-metformin-and-diverse-compounds-that-alleviate-accelerated-aging-defects-in-hutchinson-gilford-progeria-syndrome
#13
Jahahreeh Finley
Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder characterized by an accelerated aging phenotype that typically leads to death via stroke or myocardial infarction at approximately 14.6 years of age. Most cases of HGPS have been linked to the extensive use of a cryptic splice donor site located in the LMNA gene due to a de novo mutation, generating a truncated and toxic protein known as progerin. Progerin accumulation in the nuclear membrane and within the nucleus distorts the nuclear architecture and negatively effects nuclear processes including DNA replication and repair, leading to accelerated cellular aging and premature senescence...
September 2018: Medical Hypotheses
https://www.readbyqxmd.com/read/30027462/intermediate-filaments-in-cardiomyopathy
#14
REVIEW
Mary Tsikitis, Zoi Galata, Manolis Mavroidis, Stelios Psarras, Yassemi Capetanaki
Intermediate filament (IF) proteins are critical regulators in health and disease. The discovery of hundreds of mutations in IF genes and posttranslational modifications has been linked to a plethora of human diseases, including, among others, cardiomyopathies, muscular dystrophies, progeria, blistering diseases of the epidermis, and neurodegenerative diseases. The major IF proteins that have been linked to cardiomyopathies and heart failure are the muscle-specific cytoskeletal IF protein desmin and the nuclear IF protein lamin, as a subgroup of the known desminopathies and laminopathies, respectively...
August 2018: Biophysical Reviews
https://www.readbyqxmd.com/read/30026603/foxm1-repression-during-human-aging-leads-to-mitotic-decline-and-aneuploidy-driven-full-senescence
#15
Joana Catarina Macedo, Sara Vaz, Bjorn Bakker, Rui Ribeiro, Petra Lammigje Bakker, Jose Miguel Escandell, Miguel Godinho Ferreira, René Medema, Floris Foijer, Elsa Logarinho
Aneuploidy, an abnormal chromosome number, has been linked to aging and age-associated diseases, but the underlying molecular mechanisms remain unknown. Here we show, through direct live-cell imaging of young, middle-aged, and old-aged primary human dermal fibroblasts, that aneuploidy increases with aging due to general dysfunction of the mitotic machinery. Increased chromosome mis-segregation in elderly mitotic cells correlates with an early senescence-associated secretory phenotype (SASP) and repression of Forkhead box M1 (FoxM1), the transcription factor that drives G2/M gene expression...
July 19, 2018: Nature Communications
https://www.readbyqxmd.com/read/30013813/effective-pain-control-in-hutchinson-gilford-progeria-syndrome
#16
Muhammad Hassan Majeed, Muhammad Ubaidulhaq, William E Gusa
No abstract text is available yet for this article.
June 1, 2018: Innovations in Clinical Neuroscience
https://www.readbyqxmd.com/read/30012702/permanently-farnesylated-prelamin-a-progeria-and-atherosclerosis
#17
EDITORIAL
Howard J Worman, Susan Michaelis
No abstract text is available yet for this article.
July 17, 2018: Circulation
https://www.readbyqxmd.com/read/30003010/erythrocyte-senescence-in-a-model-of-rat-displaying-hutchinson-gilford-progeria-syndrome
#18
Manoj Kumar Chaudhary, Syed Ibrahim Rizvi
Background: Increased oxidative stress is a major cause of aging and age-related diseases. Erythrocytes serve as good model for aging studies. Dihydrotachysterol is known to induce premature aging feature in rats mimicking Hutchinson-Gilford progeria syndrome. Aim: In the present study, attempts have been made to explore the differential response of young and senescent erythrocytes separated by density gradient centrifugation from accelerated senescence model of rats mimicking Hutchinson-Gilford progeria syndrome and naturally aged rats...
2018: Analytical Cellular Pathology (Amsterdam)
https://www.readbyqxmd.com/read/30001457/diminished-canonical-%C3%AE-catenin-signaling-during-osteoblast-differentiation-contributes-to-osteopenia-in-progeria
#19
Ji Young Choi, Jim K Lai, Zheng-Mei Xiong, Margaret Ren, Megan C Moorer, Joseph P Stains, Kan Cao
Patients with Hutchinson-Gilford progeria syndrome (HGPS) have low bone mass and an atypical skeletal geometry that manifests in a high risk of fractures. Using both in vitro and in vivo models of HGPS, we demonstrate that defects in the canonical WNT/β-catenin pathway, seemingly at the level of the efficiency of nuclear import of β-catenin, impair osteoblast differentiation and that restoring β-catenin activity rescues osteoblast differentiation and significantly improves bone mass. Specifically, we show that HGPS patient-derived iPSCs display defects in osteoblast differentiation, characterized by a decreased alkaline phosphatase activity and mineralizing capacity...
July 12, 2018: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/29970603/inhibition-of-the-acetyltransferase-nat10-normalizes-progeric-and-aging-cells-by-rebalancing-the-transportin-1-nuclear-import-pathway
#20
Delphine Larrieu, Emmanuelle Viré, Samuel Robson, Sophia Y Breusegem, Tony Kouzarides, Stephen P Jackson
Hutchinson-Gilford progeria syndrome (HGPS) is an incurable premature aging disease. Identifying deregulated biological processes in HGPS might thus help define novel therapeutic strategies. Fibroblasts from HGPS patients display defects in nucleocytoplasmic shuttling of the GTP-bound form of the small GTPase Ran (RanGTP), which leads to abnormal transport of proteins into the nucleus. We report that microtubule stabilization in HGPS cells sequestered the nonclassical nuclear import protein Transportin-1 (TNPO1) in the cytoplasm, thus affecting the nuclear localization of its cargo, including the nuclear pore protein NUP153...
July 3, 2018: Science Signaling
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