Anesthetic management of a child with Hutchinson–Gilford progeria syndrome by Sunil Rajan, Priyanka Joseph, Gayathri Sreekumar, Maharnab Bhuyan

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Unprecedented report: First female monozygotic twins as carriers of Hutchinson-Gilford progeria syndrome by Nelson Carlos Reis-Filho, Thays Karolyne Ponte Prado Aguiar, Dafnin Lima Souza Ramos, Bruna Mariah Martins Müller, Isadora Sousa Oliveira, Felipe Augusto Cerni, Manuela Berto Pucca

Subjects: “…Hutchinson-Gilford Progeria syndrome…”
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Disease pathogenicity in Hutchinson–Gilford progeria syndrome mice: insights from lung-associated alterations by Jingjing Wang, Yuelin Guan, Yue Wang, Junyi Tan, Zhongkai Cao, Yuhan Ding, Langping Gao, Haidong Fu, Xiangjun Chen, Jianyu Lin, Ning Shen, Xudong Fu, Fangqin Wang, Jianhua Mao, Lidan Hu

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The accumulation of progerin underlies the loss of aortic smooth muscle cells in Hutchinson-Gilford progeria syndrome by Paul H. Kim, Joonyoung R. Kim, Patrick J. Heizer, Hyesoo Jung, Yiping Tu, Ashley Presnell, Julia Scheithauer, Rachel G. Yu, Stephen G. Young, Loren G. Fong

“…Abstract Hutchinson-Gilford progeria syndrome (HGPS) is caused by progerin, an internally truncated prelamin A that does not undergo the ZMPSTE24 processing step that releases prelamin A’s farnesylated carboxyl terminus; consequently, progerin remains farnesylated. …”
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Attenuated Nuclear Tension Regulates Progerin‐Induced Mechanosensitive Nuclear Wrinkling and Chromatin Remodeling by Ji‐Eun Park, Juhyeon Jo, Kun Xu, Sun‐Ah Lee, Seong‐Beom Han, YigJi Lee, Won‐Ki Cho, Bo Li, Soo Hyun Kim, Dong‐Hwee Kim

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The decision‐making process and criteria in selecting candidate drugs for progeria clinical trials by Leslie B Gordon, Mark W Kieran, Monica E Kleinman, Tom Misteli

“…Abstract Hutchinson–Gilford progeria syndrome (progeria) is an extremely rare premature aging disease with a population prevalence of 1 in 20 million. …”
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Radiological Diagnosis of a Rare Premature Aging Genetic Disorder: Progeria (Hutchinson-Gilford Syndrome) by Haji Mohammed Nazir, Akshiitha Ramesh Baabhu, Yuvaraj Muralidharan, Seena Cheppala Rajan

“…Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare disease with a combination of short stature, bone abnormalities, premature ageing, and skin changes. …”
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Altered Nuclear Functions in Progeroid Syndromes: a Paradigm for Aging Research by Baomin Li, Sonali Jog, Jose Candelario, Sita Reddy, Lucio Comai

“…Here we review recent findings on the molecular basis of two progeroid diseases, Werner syndrome (WS) and Hutchinson-Gilford progeria syndrome (HGPS), and highlight functional connections to cellular processes that may contribute to normal aging.…”
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Endoplasmic reticulum stress at the crossroads of progeria and atherosclerosis by Elisa Di Pasquale, Gianluigi Condorelli

“…Abstract Hutchinson–Gilford progeria syndrome (HGPS) is a rare pathology caused by a specific mutation (c.1824C>T; p.G608G) in the LMNA gene (Eriksson et al, 2003). …”
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Mandibuloacral Dysplasia Caused by LMNA Mutations and Uniparental Disomy by Shaochun Bai, Anthony Lozada, Marilyn C. Jones, Harry C. Dietz, Melissa Dempsey, Soma Das

“…Mandibuloacral dysplasia (MAD) is a rare autosomal recessive disorder characterized by postnatal growth retardation, craniofacial anomalies, skeletal malformations, and mottled cutaneous pigmentation. Hutchinson-Gilford Progeria Syndrome (HGPS) is characterized by the clinical features of accelerated aging in childhood. …”
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Involvement of NRF2 and AMPK signaling in aging and progeria: a digest by Eleni Petsouki, Vasileios Gerakopoulos, Despoina D. Gianniou, Elke H. Heiss, Ioannis P. Trougakos

“…This narrative short review zooms in the current understanding of NRF2 and AMPK signaling, alone or in concert, in aging and Hutchinson–Gilford Progeria Syndrome (HGPS), a genetic disorder characterized by premature aging.…”
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Role of Lysophosphatidic Acid in Neurological Diseases: From Pathophysiology to Therapeutic Implications by Simona Dedoni, Valeria Avdoshina, Maria C. Olianas, Pierluigi Onali

“…This review synthesizes evidence linking LPA’s function in neurological diseases such as traumatic brain injury, spinal cord injury, cerebellar ataxia, cerebral ischemia, seizures, Huntington’s disease, amyotrophic lateral sclerosis, Hutchinson-Gilford progeria syndrome, autism, migraine, and human immunodeficiency virus (HIV)-associated complications Despite recent advances, the specific mechanisms underlying LPA’s actions in various neurological disorders remain unknown, and further research is needed to understand the distinct roles of LPA across multiple disease conditions, as well as to investigate the therapeutic potential of targeting LPA receptors in these pathologies. …”
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MG132‐induced progerin clearance is mediated by autophagy activation and splicing regulation by Karim Harhouri, Claire Navarro, Danielle Depetris, Marie‐Geneviève Mattei, Xavier Nissan, Pierre Cau, Annachiara De Sandre‐Giovannoli, Nicolas Lévy

“…Abstract Hutchinson–Gilford progeria syndrome (HGPS) is a lethal premature and accelerated aging disease caused by a de novo point mutation in LMNA encoding A‐type lamins. …”
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Progerin accelerates atherosclerosis by inducing endoplasmic reticulum stress in vascular smooth muscle cells by Magda R Hamczyk, Ricardo Villa‐Bellosta, Víctor Quesada, Pilar Gonzalo, Sandra Vidak, Rosa M Nevado, María J Andrés‐Manzano, Tom Misteli, Carlos López‐Otín, Vicente Andrés

“…Abstract Hutchinson–Gilford progeria syndrome (HGPS) is a rare genetic disorder caused by progerin, a mutant lamin A variant. …”
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The Senolytic Drug Fisetin Attenuates Bone Degeneration in the Zmpste24−/− Progeria Mouse Model by William S. Hambright, Xiaodong Mu, Xueqin Gao, Ping Guo, Yohei Kawakami, John Mitchell, Michael Mullen, Anna-Laura Nelson, Chelsea Bahney, Haruki Nishimura, Justin Hellwinkel, Andrew Eck, Johnny Huard

“…Here, we demonstrate the efficacy of senolytic drugs (dasatinib, quercetin, and fisetin) to improve age-associated degeneration in bone using the Zmpste24−/− (Z24−/−) progeria murine system for Hutchinson–Gilford progeria syndrome (HGPS). We found that the combination of dasatinib plus quercetin could not significantly mitigate trabecular bone loss although fisetin administration could reduce bone density loss in the accelerated aging Z24−/− model. …”
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Mesenchymal Stem Cell Therapy for Hutchinson–Gilford Progeria: Improvements in Arterial Stiffness and Bone Mineral Density in a Single Case by Eun-Young Joo, Ji-Sun Park, Hyun-Tae Shin, Myungji Yoo, Su-Jin Kim, Ji-Eun Lee, Gwang-Seong Choi

“…Background/Objectives: Hutchinson–Gilford progeria syndrome (HGPS) is a rare genetic disorder that cause premature aging due to LMNA mutations and progerin accumulation. …”
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Inhibition of the NLRP3 inflammasome improves lifespan in animal murine model of Hutchinson–Gilford Progeria by Alvaro González‐Dominguez, Raúl Montañez, Beatriz Castejón‐Vega, Jéssica Nuñez‐Vasco, Débora Lendines‐Cordero, Chun Wang, Gabriel Mbalaviele, José M Navarro‐Pando, Elísabet Alcocer‐Gómez, Mario D Cordero

“…Abstract Inflammation is a hallmark of aging and accelerated aging syndromes such as Hutchinson–Gilford progeria syndrome (HGPS). In this study, we present evidence of increased expression of the components of the NLRP3 inflammasome pathway in HGPS skin fibroblasts, an outcome that was associated with morphological changes of the nuclei of the cells. …”
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Characterization of the craniofacial abnormalities of the homozygous G608G progeria mouse model by Indeevar Beeram, Maria Belen Cubria, Pramod Kamalapathy, Diana Yeritsyan, Amanda J. Dubose, Ahmad Hedayatzadeh Razavi, Nazanin Nafisi, Michael R. Erdos, Brian D. Snyder, Brian D. Snyder, Wayne A. Cabral, Francis S. Collins, Ara Nazarian, Ara Nazarian

“…IntroductionHutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic condition characterized by premature aging, impacting multiple organ systems, including cardiovascular, musculoskeletal, and integumentary. …”
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