Metabolic Dysfunction in Hutchinson–Gilford Progeria Syndrome

Development of a CRISPR/Cas9-based therapy for Hutchinson–Gilford progeria syndrome

(2019) Olaya Santiago-Fernández et al.   NATURE MEDICINE

Single-dose CRISPR–Cas9 therapy extends lifespan of mice with Hutchinson–Gilford progeria syndrome

(2019) Ergin Beyret et al.   NATURE MEDICINE

The Cutting Edge: The Role of mTOR Signaling in Laminopathies

(2019) Francesca Chiarini et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Progerin accelerates atherosclerosis by inducing endoplasmic reticulum stress in vascular smooth muscle cells

(2019) Magda R Hamczyk et al.   EMBO Molecular Medicine

Vascular smooth muscle cell‐specific progerin expression in a mouse model of Hutchinson–Gilford progeria syndrome promotes arterial stiffness: Therapeutic effect of dietary nitrite

(2019) Lara del Campo et al.   AGING CELL

Rapamycin rescues age-related changes in muscle-derived stem/progenitor cells from progeroid mice

(2019) Yohei Kawakami et al.   Molecular Therapy-Methods & Clinical Development

Healthspan and lifespan extension by fecal microbiota transplantation into progeroid mice

(2019) Clea Bárcena et al.   NATURE MEDICINE

Impaired mitochondrial respiration in human carotid plaque atherosclerosis: A potential role for Pink1 in vascular smooth muscle cell energetics

(2018) Craig K. Docherty et al.   ATHEROSCLEROSIS

Vascular Smooth Muscle–Specific Progerin Expression Accelerates Atherosclerosis and Death in a Mouse Model of Hutchinson-Gilford Progeria Syndrome

(2018) Magda R. Hamczyk et al.   CIRCULATION

Pubertal Progression in Female Adolescents with Progeria

(2018) Maya Mundkur Greer et al.   Journal of Pediatric and Adolescent Gynecology

Survey of plasma proteins in children with progeria pre-therapy and on-therapy with lonafarnib

(2018) Leslie B Gordon et al.   PEDIATRIC RESEARCH

Everolimus rescues multiple cellular defects in laminopathy-patient fibroblasts

(2018) Amanda J. DuBose et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Targeting of NAT10 enhances healthspan in a mouse model of human accelerated aging syndrome

(2018) Gabriel Balmus et al.   Nature Communications

A Cell-Intrinsic Interferon-like Response Links Replication Stress to Cellular Aging Caused by Progerin

(2018) Ray Kreienkamp et al.   Cell Reports

Boosting ATM activity alleviates aging and extends lifespan in a mouse model of progeria

(2018) Minxian Qian et al.   eLife

Cardiac Abnormalities in Patients With Hutchinson-Gilford Progeria Syndrome

(2018) Ashwin Prakash et al.   JAMA Cardiology

Association between insulin resistance and the development of cardiovascular disease

(2018) Valeska Ormazabal et al.   Cardiovascular Diabetology

Methionine Restriction Extends Lifespan in Progeroid Mice and Alters Lipid and Bile Acid Metabolism

(2018) Clea Bárcena et al.   Cell Reports

Doubled lifespan and patient‐like pathologies in progeria mice fed high‐fat diet

(2018) Ray Kreienkamp et al.   AGING CELL

Endothelial progerin expression causes cardiovascular pathology through an impaired mechanoresponse

(2018) Selma Osmanagic-Myers et al.   JOURNAL OF CLINICAL INVESTIGATION

Animal models of atherosclerosis

(2017) Besa Emini Veseli et al.   EUROPEAN JOURNAL OF PHARMACOLOGY

Metformin alleviates ageing cellular phenotypes in Hutchinson-Gilford progeria syndrome dermal fibroblasts

(2017) Seul-Ki Park et al.   EXPERIMENTAL DERMATOLOGY

Progerin-Induced Replication Stress Facilitates Premature Senescence in Hutchinson-Gilford Progeria Syndrome

(2017) Keith Wheaton et al.   MOLECULAR AND CELLULAR BIOLOGY

High incidence of coding gene mutations in mitochondrial DNA in esophageal cancer

(2017) Zong-Wen Liu et al.   Molecular Medicine Reports

Shared molecular and cellular mechanisms of premature ageing and ageing-associated diseases

(2017) Nard Kubben et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Metformin and cancer: An existing drug for cancer prevention and therapy (Review)

(2017) Fuming Zi et al.   Oncology Letters

Nucleolar expansion and elevated protein translation in premature aging

(2017) Abigail Buchwalter et al.   Nature Communications

Intermittent treatment with farnesyltransferase inhibitor and sulforaphane improves cellular homeostasis in Hutchinson-Gilford progeria fibroblasts

(2017) Diana Gabriel et al.   Oncotarget

The protective role of estrogen and estrogen receptors in cardiovascular disease and the controversial use of estrogen therapy

(2017) Andrea Iorga et al.   Biology of Sex Differences

Temsirolimus Partially Rescues the Hutchinson-Gilford Progeria Cellular Phenotype

(2017) Diana Gabriel et al.   PLoS One

Potential therapeutic effects of the MTOR inhibitors for preventing ageing and progeria-related disorders

(2016) Camilla Evangelisti et al.   BRITISH JOURNAL OF CLINICAL PHARMACOLOGY

In Vivo Amelioration of Age-Associated Hallmarks by Partial Reprogramming

(2016) Alejandro Ocampo et al.   CELL

Repression of the Antioxidant NRF2 Pathway in Premature Aging

(2016) Nard Kubben et al.   CELL

Metformin as a Tool to Target Aging

(2016) Nir Barzilai et al.   Cell Metabolism

Clinical Trial of the Protein Farnesylation Inhibitors Lonafarnib, Pravastatin, and Zoledronic Acid in Children With Hutchinson-Gilford Progeria SyndromeClinical Perspective

(2016) Leslie B. Gordon et al.   CIRCULATION

Modulation of LMNA splicing as a strategy to treat prelamin A diseases

(2016) John M. Lee et al.   JOURNAL OF CLINICAL INVESTIGATION

mTOR signaling plays a critical role in the defects observed in muscle-derived stem/progenitor cells isolated from a murine model of accelerated aging

(2016) Koji Takayama et al.   JOURNAL OF ORTHOPAEDIC RESEARCH

Vitamin D receptor signaling improves Hutchinson-Gilford progeria syndrome cellular phenotypes

(2016) Ray Kreienkamp et al.   Oncotarget

Methylene blue alleviates nuclear and mitochondrial abnormalities in progeria

(2015) Zheng-Mei Xiong et al.   AGING CELL

Restoring SIRT6 Expression in Hutchinson-Gilford Progeria Syndrome Cells Impedes Premature Senescence and Formation of Dysmorphic Nuclei

(2015) Helal Endisha et al.   PATHOBIOLOGY

All-trans retinoic acid and rapamycin normalize Hutchinson Gilford progeria fibroblast phenotype

(2015) Camilla Pellegrini et al.   Oncotarget

Sulforaphane enhances progerin clearance in Hutchinson-Gilford progeria fibroblasts

(2014) Diana Gabriel et al.   AGING CELL

Progeria: A Paradigm for Translational Medicine

(2014) Leslie B. Gordon et al.   CELL

Geroscience: Linking Aging to Chronic Disease

(2014) Brian K. Kennedy et al.   CELL

Impact of Farnesylation Inhibitors on Survival in Hutchinson-Gilford Progeria Syndrome

(2014) Leslie B. Gordon et al.   CIRCULATION

Risk of Cardiac Arrhythmias During Hypoglycemia in Patients With Type 2 Diabetes and Cardiovascular Risk

(2014) Elaine Chow et al.   DIABETES

Antagonistic functions of LMNA isoforms in energy expenditure and lifespan

(2014) I. C. Lopez-Mejia et al.   EMBO REPORTS

Making new contacts: the mTOR network in metabolism and signalling crosstalk

(2014) Mitsugu Shimobayashi et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Initial Cutaneous Manifestations of Hutchinson-Gilford Progeria Syndrome

(2014) Jillian F. Rork et al.   PEDIATRIC DERMATOLOGY

Chemical Inhibition of NAT10 Corrects Defects of Laminopathic Cells

(2014) D. Larrieu et al.   SCIENCE

Transformation Resistance in a Premature Aging Disorder Identifies a Tumor-Protective Function of BRD4

(2014) Patricia Fernandez et al.   Cell Reports

Defective Extracellular Pyrophosphate Metabolism Promotes Vascular Calcification in a Mouse Model of Hutchinson-Gilford Progeria Syndrome That Is Ameliorated on Pyrophosphate Treatment

(2013) Ricardo Villa-Bellosta et al.   CIRCULATION

Lamins are rapamycin targets that impact human longevity: a study in centenarians

(2013) G. Lattanzi et al.   JOURNAL OF CELL SCIENCE

Identification of mitochondrial dysfunction in Hutchinson–Gilford progeria syndrome through use of stable isotope labeling with amino acids in cell culture

(2013) José Rivera-Torres et al.   Journal of Proteomics

Targeting Isoprenylcysteine Methylation Ameliorates Disease in a Mouse Model of Progeria

(2013) M. X. Ibrahim et al.   SCIENCE

mTOR Signaling in Growth Control and Disease

(2012) Mathieu Laplante et al.   CELL

Resveratrol Rescues SIRT1-Dependent Adult Stem Cell Decline and Alleviates Progeroid Features in Laminopathy-Based Progeria

(2012) Baohua Liu et al.   Cell Metabolism

The Critical Role of Metabolic Pathways in Aging

(2012) N. Barzilai et al.   DIABETES

Correlated alterations in genome organization, histone methylation, and DNA-lamin A/C interactions in Hutchinson-Gilford progeria syndrome

(2012) R. P. McCord et al.   GENOME RESEARCH

Sirtuin 1 and Sirtuin 3: Physiological Modulators of Metabolism

(2012) Ruben Nogueiras et al.   PHYSIOLOGICAL REVIEWS

Insulin Resistance and Risk of Incident Cardiovascular Events in Adults without Diabetes: Meta-Analysis

(2012) Karin B. Gast et al.   PLoS One

Regulation of prelamin A but not lamin C by miR-9, a brain-specific microRNA

(2012) H.-J. Jung et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Rapamycin Reverses Elevated mTORC1 Signaling in Lamin A/C-Deficient Mice, Rescues Cardiac and Skeletal Muscle Function, and Extends Survival

(2012) F. J. Ramos et al.   Science Translational Medicine

Mechanisms of Premature Vascular Aging in Children With Hutchinson-Gilford Progeria Syndrome

(2011) Marie Gerhard-Herman et al.   HYPERTENSION

Hutchinson-gilford progeria is a skeletal dysplasia

(2011) Catherine M Gordon et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Splicing-Directed Therapy in a New Mouse Model of Human Accelerated Aging

(2011) F. G. Osorio et al.   Science Translational Medicine

Rapamycin Reverses Cellular Phenotypes and Enhances Mutant Protein Clearance in Hutchinson-Gilford Progeria Syndrome Cells

(2011) K. Cao et al.   Science Translational Medicine

Cardiovascular Pathology in Hutchinson-Gilford Progeria: Correlation With the Vascular Pathology of Aging

(2010) Michelle Olive et al.   ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY

The Histone Deacetylase Sirt6 Regulates Glucose Homeostasis via Hif1α

(2010) Lei Zhong et al.   CELL

Defective DSB repair correlates with abnormal nuclear morphology and is improved with FTI treatment in Hutchinson-Gilford progeria syndrome fibroblasts

(2010) Dan Constantinescu et al.   EXPERIMENTAL CELL RESEARCH

Novel roles for A-type lamins in telomere biology and the DNA damage response pathway

(2009) Ignacio Gonzalez-Suarez et al.   EMBO JOURNAL

Telomere length in Hutchinson-Gilford Progeria Syndrome

(2009) Michelle L. Decker et al.   MECHANISMS OF AGEING AND DEVELOPMENT

Effect of progerin on the accumulation of oxidized proteins in fibroblasts from Hutchinson Gilford progeria patients

(2009) Gabriela Viteri et al.   MECHANISMS OF AGEING AND DEVELOPMENT

Leptin and Cardiovascular Disease

(2008) Kwang Kon Koh et al.   CIRCULATION

Phenotype and Course of Hutchinson–Gilford Progeria Syndrome

(2008) Melissa A. Merideth et al.   NEW ENGLAND JOURNAL OF MEDICINE

A farnesyltransferase inhibitor prevents both the onset and late progression of cardiovascular disease in a progeria mouse model

(2008) B. C. Capell et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA