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

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

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

Deficiency in ZMPSTE24 and resulting farnesyl–prelamin A accumulation only modestly affect mouse adipose tissue stores

(2020) Patrick J. Heizer et al.   JOURNAL OF LIPID RESEARCH

Evaluation of musculoskeletal phenotype of the G608G progeria mouse model with lonafarnib, pravastatin, and zoledronic acid as treatment groups

(2020) Maria B. Cubria et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Targeting RAS‐converting enzyme 1 overcomes senescence and improves progeria‐like phenotypes of ZMPSTE24 deficiency

(2020) Haidong Yao et al.   AGING CELL

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

Postnatal development of mice with combined genetic depletions of lamin A/C, emerin and lamina-associated polypeptide 1

(2019) Yuexia Wang et al.   HUMAN MOLECULAR GENETICS

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

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

Proteomic Evidence of Biological Ageing in a Child with a Compound Heterozygous ZMPSTE24 Mutation

(2018) Angela K Lucas‐Herald et al.   Proteomics Clinical Applications

ZMPSTE24 defends against influenza and other pathogenic viruses

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

The Protease Ste24 Clears Clogged Translocons

(2016) Tslil Ast et al.   CELL

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

LMNA missense mutations causing familial partial lipodystrophy do not lead to an accumulation of prelamin A

(2016) Yiping Tu et al.   Nucleus

LMNA missense mutations causing familial partial lipodystrophy do not lead to an accumulation of prelamin A

(2016) Yiping Tu et al.   Nucleus

Dysregulated interactions between lamin A and SUN1 induce abnormalities in the nuclear envelope and endoplasmic reticulum in progeric laminopathies

(2014) Z.-J. Chen et al.   JOURNAL OF CELL SCIENCE

Assessing Functional Performance in the Mdx Mouse Model

(2014) Annemieke Aartsma-Rus et al.   Jove-Journal of Visualized Experiments

Prelamin A Accelerates Vascular Calcification Via Activation of the DNA Damage Response and Senescence-Associated Secretory Phenotype in Vascular Smooth Muscle Cells

(2013) Yiwen Liu et al.   CIRCULATION RESEARCH

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

Targeting Isoprenylcysteine Methylation Ameliorates Disease in a Mouse Model of Progeria

(2013) M. X. Ibrahim et al.   SCIENCE

Mouse models of laminopathies

(2012) Haoyue Zhang et al.   AGING CELL

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

(2012) Jemima Barrowman et al.   HUMAN MOLECULAR GENETICS

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

An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria

(2010) B. S. J. Davies et al.   HUMAN MOLECULAR GENETICS

The Genetic Landscape of a Cell

(2010) M. Costanzo et al.   SCIENCE

The Posttranslational Processing of Prelamin A and Disease

(2009) Brandon S.J. Davies et al.   Annual Review of Genomics and Human Genetics

Severe mandibuloacral dysplasia caused by novel compound heterozygous ZMPSTE24 mutations in two Japanese siblings

(2009) Y Miyoshi et al.   CLINICAL GENETICS

Accelerated Features of Age-Related Bone Loss in Zmpste24 Metalloproteinase-Deficient Mice

(2009) D. Rivas et al.   JOURNALS OF GERONTOLOGY SERIES A-BIOLOGICAL SCIENCES AND MEDICAL SCIENCES

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

(2009) M. C. Jonikas et al.   SCIENCE

Premature aging in mice activates a systemic metabolic response involving autophagy induction

(2008) Guillermo Mariño et al.   HUMAN MOLECULAR GENETICS

Combined treatment with statins and aminobisphosphonates extends longevity in a mouse model of human premature aging

(2008) Ignacio Varela et al.   NATURE MEDICINE

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