Prelamin A and ZMPSTE24 in premature and physiological aging

Hallmarks of aging: An expanding universe

(2023) Carlos López-Otín et al.   CELL

Progerin-expressing endothelial cells are unable to adapt to shear stress

(2022) Brooke E. Danielsson et al.   BIOPHYSICAL JOURNAL

Abolishing the prelamin A ZMPSTE24 cleavage site leads to progeroid phenotypes with near-normal longevity in mice

(2022) Yuexia Wang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Lamin A to Z in normal aging

(2022) Stanley R. Primmer et al.   Aging-US

FDA approval summary for lonafarnib (Zokinvy) for the treatment of Hutchinson-Gilford progeria syndrome and processing-deficient progeroid laminopathies

(2022) Mari Suzuki et al.   GENETICS IN MEDICINE

A small-molecule ICMT inhibitor delays senescence of Hutchinson-Gilford progeria syndrome cells

(2021) Xue Chen et al.   eLife

LINC complex regulation of genome organization and function

(2021) Xianrong Wong et al.   CURRENT OPINION IN GENETICS & DEVELOPMENT

Molecular and Cellular Mechanisms Driving Cardiovascular Disease in Hutchinson-Gilford Progeria Syndrome: Lessons Learned from Animal Models

(2021) Ignacio Benedicto et al.   Cells

Genetic reduction of mTOR extends lifespan in a mouse model of Hutchinson‐Gilford Progeria syndrome

(2021) Wayne A. Cabral et al.   AGING CELL

Mechanisms of angiogenic incompetence in Hutchinson–Gilford progeria via downregulation of endothelial NOS

(2021) Yantenew G. Gete et al.   AGING CELL

Molecular Basis of Bone Aging

(2020) Addolorata Corrado et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Towards delineating the chain of events that cause premature senescence in the accelerated aging syndrome Hutchinson–Gilford progeria (HGPS)

(2020) Oliver Dreesen   BIOCHEMICAL SOCIETY TRANSACTIONS

The ER-associated protease Ste24 prevents N-terminal signal peptide-independent translocation into the endoplasmic reticulum in Saccharomyces cerevisiae

(2020) Akira Hosomi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Overlapping function of Hrd1 and Ste24 in translocon quality control provides robust channel surveillance

(2020) Avery M. Runnebohm et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Premature Vascular Aging with Features of Plaque Vulnerability in an Atheroprone Mouse Model of Hutchinson–Gilford Progeria Syndrome with Ldlr Deficiency

(2020) Rosa M. Nevado et al.   Cells

Imbalanced nucleocytoskeletal connections create common polarity defects in progeria and physiological aging

(2019) Wakam Chang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Osteoporosis

(2019) Juliet E Compston et al.   LANCET

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

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

The Driving Force: Nuclear Mechanotransduction in Cellular Function, Fate, and Disease

(2019) Melanie Maurer et al.   Annual Review of Biomedical Engineering

Long term breeding of the Lmna G609G progeric mouse: Characterization of homozygous and heterozygous models

(2019) Anna Zaghini et al.   EXPERIMENTAL GERONTOLOGY

Aging in the Cardiovascular System: Lessons from Hutchinson-Gilford Progeria Syndrome

(2018) Magda R. Hamczyk et al.   Annual Review of Physiology

Translocon Declogger Ste24 Protects against IAPP Oligomer-Induced Proteotoxicity

(2018) Can Kayatekin et al.   CELL

Permanently Farnesylated Prelamin A, Progeria, and Atherosclerosis

(2018) Howard J. Worman et al.   CIRCULATION

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

ZMPSTE24 missense mutations that cause progeroid diseases decrease prelamin A cleavage activity and/or protein stability

(2018) Eric D. Spear et al.   Disease Models & Mechanisms

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

(2018) Ray Kreienkamp et al.   Cell Reports

Disrupting the LINC complex in smooth muscle cells reduces aortic disease in a mouse model of Hutchinson-Gilford progeria syndrome

(2018) Paul H. Kim et al.   Science Translational Medicine

Endothelial progerin expression causes cardiovascular pathology through an impaired mechanoresponse

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

ZMPSTE24 defends against influenza and other pathogenic viruses

(2017) Bishi Fu et al.   JOURNAL OF EXPERIMENTAL MEDICINE

The molecular architecture of lamins in somatic cells

(2017) Yagmur Turgay et al.   NATURE

The structure of lamin filaments in somatic cells as revealed by cryo-electron tomography

(2017) Y. Turgay et al.   Nucleus

The structure of lamin filaments in somatic cells as revealed by cryo-electron tomography

(2017) Y. Turgay et al.   Nucleus

The Protease Ste24 Clears Clogged Translocons

(2016) Tslil Ast et al.   CELL

Repression of the Antioxidant NRF2 Pathway in Premature Aging

(2016) Nard Kubben et al.   CELL

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

A mutation abolishing the ZMPSTE24 cleavage site in prelamin A causes a progeroid disorder

(2016) Yuexia Wang et al.   JOURNAL OF CELL SCIENCE

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

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

Protein prenylation: unique fats make their mark on biology

(2016) Mei Wang et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Human CaaX protease ZMPSTE24 expressed in yeast: Structure and inhibition by HIV protease inhibitors

(2016) Kathleen M. Clark et al.   PROTEIN SCIENCE

The protease inhibitors and HIV-associated bone loss

(2016) Caitlin A. Moran et al.   Current Opinion in HIV and AIDS

Accelerated aging syndromes, are they relevant to normal human aging?

(2016) Oliver Dreesen et al.   Aging-US

Methylene blue alleviates nuclear and mitochondrial abnormalities in progeria

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

Lamins: Nuclear Intermediate Filament Proteins with Fundamental Functions in Nuclear Mechanics and Genome Regulation

(2015) Yosef Gruenbaum et al.   Annual Review of Biochemistry

DNA repair defects and genome instability in Hutchinson–Gilford Progeria Syndrome

(2015) Susana Gonzalo et al.   CURRENT OPINION IN CELL BIOLOGY

Accessorizing and anchoring the LINC complex for multifunctionality

(2015) Wakam Chang et al.   JOURNAL OF CELL BIOLOGY

Progeria: A Paradigm for Translational Medicine

(2014) Leslie B. Gordon et al.   CELL

Nuclear localization signal deletion mutants of lamin A and progerin reveal insights into lamin A processing and emerin targeting

(2014) Di Wu et al.   Nucleus

HIV protease inhibitors and onset of cardiovascular diseases: A central role for oxidative stress and dysregulation of the ubiquitin–proteasome system

(2013) Kathleen M.S.E. Reyskens et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

The Hallmarks of Aging

(2013) Carlos López-Otín et al.   CELL

Nuclear Positioning

(2013) Gregg G. Gundersen et al.   CELL

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

New ZMPSTE24 (FACE1) mutations in patients affected with restrictive dermopathy or related progeroid syndromes and mutation update

(2013) Claire Laure Navarro et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

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

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

A Protease for the Ages

(2013) S. Michaelis et al.   SCIENCE

The Structural Basis of ZMPSTE24-Dependent Laminopathies

(2013) A. Quigley et al.   SCIENCE

Structure of the Integral Membrane Protein CAAX Protease Ste24p

(2013) E. E. Pryor et al.   SCIENCE

Targeting Isoprenylcysteine Methylation Ameliorates Disease in a Mouse Model of Progeria

(2013) M. X. Ibrahim et al.   SCIENCE

Nuclear Lamin-A Scales with Tissue Stiffness and Enhances Matrix-Directed Differentiation

(2013) J. Swift et al.   SCIENCE

LINCing complex functions at the nuclear envelope

(2013) Andrea Rothballer et al.   Nucleus

Mouse models of laminopathies

(2012) Haoyue Zhang et al.   AGING CELL

LINC Complexes Form by Binding of Three KASH Peptides to Domain Interfaces of Trimeric SUN Proteins

(2012) Brian A. Sosa et al.   CELL

The Intersection Between Aging and Cardiovascular Disease

(2012) Brian J. North et al.   CIRCULATION RESEARCH

Human ZMPSTE24 disease mutations: residual proteolytic activity correlates with disease severity

(2012) Jemima Barrowman et al.   HUMAN MOLECULAR GENETICS

Clinical trial of a farnesyltransferase inhibitor in children with Hutchinson-Gilford progeria syndrome

(2012) L. B. Gordon et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Progerin and telomere dysfunction collaborate to trigger cellular senescence in normal human fibroblasts

(2011) Kan Cao et al.   JOURNAL OF CLINICAL INVESTIGATION

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

(2011) F. G. Osorio 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

Prelamin A Acts to Accelerate Smooth Muscle Cell Senescence and Is a Novel Biomarker of Human Vascular Aging

(2010) Cassandra D. Ragnauth et al.   CIRCULATION

Role of progerin-induced telomere dysfunction in HGPS premature cellular senescence

(2010) E. K. Benson et al.   JOURNAL OF CELL SCIENCE

The Burden of Cardiovascular Disease in the Elderly: Morbidity, Mortality, and Costs

(2009) Ali Yazdanyar et al.   CLINICS IN GERIATRIC MEDICINE

Comprehensive Characterization of Genes Required for Protein Folding in the Endoplasmic Reticulum

(2009) M. C. Jonikas et al.   SCIENCE

Increased mechanosensitivity and nuclear stiffness in Hutchinson–Gilford progeria cells: effects of farnesyltransferase inhibitors

(2008) Valerie L. R. M. Verstraeten et al.   AGING CELL

HIV-protease inhibitors block the enzymatic activity of purified Ste24p

(2008) Sarah E. Hudon et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

A Potent HIV Protease Inhibitor, Darunavir, Does Not Inhibit ZMPSTE24 or Lead to an Accumulation of Farnesyl-prelamin A in Cells

(2008) Catherine Coffinier et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Nuclear envelope defects cause stem cell dysfunction in premature-aging mice

(2008) Jesús Espada et al.   JOURNAL OF CELL BIOLOGY

Analysis of Prelamin A Biogenesis Reveals the Nucleus to be a CaaX Processing Compartment

(2008) Jemima Barrowman et al.   MOLECULAR BIOLOGY OF THE CELL

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