Ameliorating calcium homeostasis improves longevity and healthspan in progeroid and naturally aged mice

Farnesylated prelamin A induces fibroblast polarity defects in premature aging disorders by inhibiting nesprin-2-SUN2 LINC complex function – PubMed

A Long-lived Avatar for Modeling Age-Related Vascular Disease

Increased Telomere Mobility in Progeria is Restored by Isoprenylcysteine Carboxyl Methyltransferase Inhibition

Detection of nuclear STING in cultured human cells and in the normal and cancer tissues

First Generation Proteolysis Targeting Chimeras (PROTACs) for the Treatment of Progeria

Hutchinson-Gilford progeria syndrome alters the endothelial genetic response to laminar shear stress

Senescence-inhibitory Δ133p53α counteracts accelerated ageing and mortality

Selection of specific and efficient siRNAs in new cellular model for Hutchinson-Gilford progeria syndrome therapy

Generation of Nonintegrative-Induced Pluripotent Stem Cells in Hutchinson-Gilford Progeria Syndrome: Enhancing Aging Research

A longevity-associated variant of the human BPIFB4 gene prevents diastolic dysfunction in progeria mice

Manipulation of the nucleoscaffold potentiates cellular reprogramming kinetics

Transcriptional profiling of Hutchinson-Gilford Progeria patients identifies primary target pathways of progerin

Deregulated miR-145 and miR-27b in hutchinson-gilford progeria syndrome: implications for adipogenesis

Impact of miR-181a on SIRT1 Expression and Senescence in Hutchinson-Gilford Progeria Syndrome

A Quantitative High-Throughput Screen Identifies Compounds that Upregulate the p53 Isoform Δ133p53α and Inhibit Cellular Senescence

Pharmacologic activation of Δ133p53α reduces cellular senescence in progeria patients-derived cells

Patient-Derived Cortical Organoids Reveal Senescence of Neural Progenitor Cells in Hutchinson-Gilford Progeria Syndrome

Baricitinib and Lonafarnib Synergistically Target Progerin and Inflammation, Improving Lifespan and Health in Progeria Mice

Hutchinson-Gilford Progeria Syndrome

Circular RNA Telomerase Reverses Endothelial Senescence in Progeria

Adenine base editing rescues pathogenic phenotypes in tissue engineered vascular model of Hutchinson-Gilford progeria syndrome

Aged-vascular niche hinders osteogenesis of mesenchymal stem cells through paracrine repression of Wnt-axis

Aberrant migration features in primary skin fibroblasts of Huntington’s disease patients hold potential for unraveling disease progression using an image based machine learning tool

Enhancing Cellular Homeostasis: Targeted Botanical Compounds Boost Cellular Health Functions in Normal and Premature Aging Fibroblasts

The NLRP3 inhibitor Dapansutrile improves the therapeutic action of lonafarnib on progeroid mice

Progeria-based vascular model identifies networks associated with cardiovascular aging and disease

Angiopoietin-2 reverses endothelial cell dysfunction in progeria vasculature

Progerin mRNA expression in non-HGPS patients is correlated with widespread shifts in transcript isoforms

Coaching ribosome biogenesis from the nuclear periphery

Lonafarnib and everolimus reduce pathology in iPSC-derived tissue engineered blood vessel model of Hutchinson-Gilford Progeria Syndrome

Aging Model for Analyzing Drug-Induced Proarrhythmia Risks Using Cardiomyocytes Differentiated from Progeria-Patient-Derived Induced Pluripotent Stem Cells

Ghrelin delays premature aging in Hutchinson-Gilford progeria syndrome

Perturbed actin cap as a new personalized biomarker in primary fibroblasts of Huntington’s disease patients

Plasma Progerin in Patients With Hutchinson-Gilford Progeria Syndrome: Immunoassay Development and Clinical Evaluation

Impact of Combined Baricitinib and FTI Treatment on Adipogenesis in Hutchinson-Gilford Progeria Syndrome and Other Lipodystrophic Laminopathies

Lonafarnib improves cardiovascular function and survival in a mouse model of Hutchinson-Gilford progeria syndrome

The farnesyl transferase inhibitor (FTI) lonafarnib improves nuclear morphology in ZMPSTE24-deficient fibroblasts from patients with the progeroid disorder MAD-B

Hutchinson-Gilford progeria patient-derived cardiomyocyte model of carrying LMNA gene variant c.1824 C > T

Remodeling of the Cardiac Extracellular Matrix Proteome During Chronological and Pathological Aging

Activation of endoplasmic reticulum stress in premature aging via the inner nuclear membrane protein SUN2

Unique progerin C-terminal peptide ameliorates Hutchinson-Gilford progeria syndrome phenotype by rescuing BUBR1

Senotherapeutic peptide treatment reduces biological age and senescence burden in human skin models

Quantification of Farnesylated Progerin in Hutchinson-Gilford Progeria Patient Cells by Mass Spectrometry

SerpinE1 drives a cell-autonomous pathogenic signaling in Hutchinson-Gilford progeria syndrome

Clonal hematopoiesis is not prevalent in Hutchinson-Gilford progeria syndrome

MG132 Induces Progerin Clearance and Improves Disease Phenotypes in HGPS-like Patients’ Cells

Anti-hsa-miR-59 alleviates premature senescence associated with Hutchinson-Gilford progeria syndrome in mice

Combined alteration of lamin and nuclear morphology influences the localization of the tumor-associated factor AKTIP

Establishment and Characterization of hTERT Immortalized Hutchinson-Gilford Progeria Fibroblast Cell Lines

Impaired LEF1 Activation Accelerates iPSC-Derived Keratinocytes Differentiation in Hutchinson-Gilford Progeria Syndrome

Modelling premature cardiac aging with induced pluripotent stem cells from a hutchinson-gilford Progeria Syndrome patient

Gaussian curvature dilutes the nuclear lamina, favoring nuclear rupture, especially at high strain rate

Transcriptional profiling of Hutchinson-Gilford Progeria syndrome fibroblasts reveals deficits in mesenchymal stem cell commitment to differentiation related to early events in endochondral ossification

Impact of MnTBAP and Baricitinib Treatment on Hutchinson-Gilford Progeria Fibroblasts

Achieving single nucleotide sensitivity in direct hybridization genome imaging

Vascular senescence in progeria: role of endothelial dysfunction

Baricitinib, a JAK-STAT Inhibitor, Reduces the Cellular Toxicity of the Farnesyltransferase Inhibitor Lonafarnib in Progeria Cells

A targeted antisense therapeutic approach for Hutchinson-Gilford progeria syndrome

Self-assembly of multi-component mitochondrial nucleoids via phase separation

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

Inhibition of the NLRP3 inflammasome improves lifespan in animal murine model of Hutchinson-Gilford Progeria

Progerinin, an optimized progerin-lamin A binding inhibitor, ameliorates premature senescence phenotypes of Hutchinson-Gilford progeria syndrome

In vivo base editing rescues Hutchinson-Gilford progeria syndrome in mice

Isoprenylcysteine Carboxylmethyltransferase-Based Therapy for Hutchinson-Gilford Progeria Syndrome

Telomerase therapy reverses vascular senescence and extends lifespan in progeria mice

Impact of Progerin Expression on Adipogenesis in Hutchinson-Gilford Progeria Skin-Derived Precursor Cells

Systematic screening identifies therapeutic antisense oligonucleotides for Hutchinson-Gilford progeria syndrome

Nuclear Pore Complexes Cluster in Dysmorphic Nuclei of Normal and Progeria Cells during Replicative Senescence

iPSC-Derived Endothelial Cells Affect Vascular Function in a Tissue-Engineered Blood Vessel Model of Hutchinson-Gilford Progeria Syndrome

Direct reprogramming of human smooth muscle and vascular endothelial cells reveals defects associated with aging and Hutchinson-Gilford progeria syndrome

Phosphorylated Lamin A/C in the Nuclear Interior Binds Active Enhancers Associated with Abnormal Transcription in Progeria

Epigenetic deregulation of lamina-associated domains in Hutchinson-Gilford progeria syndrome

Chromatin and Cytoskeletal Tethering Determine Nuclear Morphology in Progerin-Expressing Cells

Peroxisomal abnormalities and catalase deficiency in Hutchinson-Gilford Progeria Syndrome

SAMMY-seq reveals early alteration of heterochromatin and deregulation of bivalent genes in Hutchinson-Gilford Progeria Syndrome

PML2-mediated thread-like nuclear bodies mark late senescence in Hutchinson-Gilford progeria syndrome

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

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

Transient introduction of human telomerase mRNA improves hallmarks of progeria cells

Inhibition of JAK-STAT Signaling with Baricitinib Reduces Inflammation and Improves Cellular Homeostasis in Progeria Cells

Dysfunction of iPSC-derived endothelial cells in human Hutchinson-Gilford progeria syndrome

Metabolomic profiling suggests systemic signatures of premature aging induced by Hutchinson-Gilford progeria syndrome

Analysis of somatic mutations identifies signs of selection during in vitro aging of primary dermal fibroblasts

Restoring extracellular matrix synthesis in senescent stem cells

Smurf2 regulates stability and the autophagic-lysosomal turnover of lamin A and its disease-associated form progerin

Progerin phosphorylation in interphase is lower and less mechanosensitive than lamin-A,C in iPS-derived mesenchymal stem cells

Diminished Canonical β-Catenin Signaling During Osteoblast Differentiation Contributes to Osteopenia in Progeria

Everolimus rescues multiple cellular defects in laminopathy-patient fibroblasts

Predicting age from the transcriptome of human dermal fibroblasts

Targeting the phospholipase A2 receptor ameliorates premature aging phenotypes

Epigenetic clock for skin and blood cells applied to Hutchinson Gilford Progeria Syndrome and ex vivo studies

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

Analyses of LMNA-negative juvenile progeroid cases confirms biallelic POLR3A mutations in Wiedemann-Rautenstrauch-like syndrome and expands the phenotypic spectrum of PYCR1 mutations

Autophagic Removal of Farnesylated Carboxy-Terminal Lamin Peptides

p53 isoforms regulate premature aging in human cells

SIRPA-Inhibited, Marrow-Derived Macrophages Engorge, Accumulate, and Differentiate in Antibody-Targeted Regression of Solid Tumors

Nucleolar expansion and elevated protein translation in premature aging

Reprogramming progeria fibroblasts re-establishes a normal epigenetic landscape

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

Progerin sequestration of PCNA promotes replication fork collapse and mislocalization of XPA in laminopathy-related progeroid syndromes

Cross-linked matrix rigidity and soluble retinoids synergize in nuclear lamina regulation of stem cell differentiation

Identification of novel PDEδ interacting proteins

Telomerase mRNA Reverses Senescence in Progeria Cells

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

Nucleoplasmic lamins define growth-regulating functions of lamina-associated polypeptide 2α in progeria cells

A novel somatic mutation achieves partial rescue in a child with Hutchinson-Gilford progeria syndrome

Progerin impairs chromosome maintenance by depleting CENP-F from metaphase kinetochores in Hutchinson-Gilford progeria fibroblasts

Temsirolimus Partially Rescues the Hutchinson-Gilford Progeria Cellular Phenotype

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

NANOG Reverses the Myogenic Differentiation Potential of Senescent Stem Cells by Restoring ACTIN Filamentous Organization and SRF-Dependent Gene Expression

Permanent farnesylation of lamin A mutants linked to progeria impairs its phosphorylation at serine 22 during interphase

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

Comparing lamin proteins post-translational relative stability using a 2A peptide-based system reveals elevated resistance of progerin to cellular degradation

Loss of H3K9me3 Correlates with ATM Activation and Histone H2AX Phosphorylation Deficiencies in Hutchinson-Gilford Progeria Syndrome

Nuclear stiffening and chromatin softening with progerin expression leads to an attenuated nuclear response to force

Lamin A Is an Endogenous SIRT6 Activator and Promotes SIRT6-Mediated DNA Repair

Insights into the role of immunosenescence during varicella zoster virus infection (shingles) in the aging cell model

Proliferation of progeria cells is enhanced by lamina-associated polypeptide 2α (LAP2α) through expression of extracellular matrix proteins

Phenotype-Dependent Coexpression Gene Clusters: Application to Normal and Premature Ageing

Methylene blue alleviates nuclear and mitochondrial abnormalities in progeria

Sulforaphane enhances progerin clearance in Hutchinson-Gilford progeria fibroblasts

Depleting the methyltransferase Suv39h1 improves DNA repair and extends lifespan in a progeria mouse model

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

Higher-order unfolding of satellite heterochromatin is a consistent and early event in cell senescence

An inhibitory role of progerin in the gene induction network of adipocyte differentiation from iPS cells

Automated image analysis of nuclear shape: what can we learn from a prematurely aged cell?

Progeria: translational insights from cell biology

A proteomic study of Hutchinson-Gilford progeria syndrome: Application of 2D-chromotography in a premature aging disease

Naïve adult stem cells from patients with Hutchinson-Gilford progeria syndrome express low levels of progerin in vivo

Comparison of constitutional and replication stress-induced genome structural variation by SNP array and mate-pair sequencing

Hydroxyurea induces de novo copy number variants in human cells

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

Rapamycin reverses cellular phenotypes and enhances mutant protein clearance in Hutchinson-Gilford progeria syndrome cells

Computational image analysis of nuclear morphology associated with various nuclear-specific aging disorders

CTP: phosphocholine cytidylyltransferase α (CCTα) and lamins alter nuclear membrane structure without affecting phosphatidylcholine synthesis

Age-dependent loss of MMP-3 in Hutchinson-Gilford progeria syndrome

Low and high expressing alleles of the LMNA gene: implications for laminopathy disease development

Stem cell depletion in Hutchinson-Gilford progeria syndrome

Defective lamin A-Rb signaling in Hutchinson-Gilford Progeria Syndrome and reversal by farnesyltransferase inhibition

Cardiovascular pathology in Hutchinson-Gilford progeria: correlation with the vascular pathology of aging

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

Replication stress induces genome-wide copy number changes in human cells that resemble polymorphic and pathogenic variants

Ageing-related chromatin defects through loss of the NURD complex

Perturbation of wild-type lamin A metabolism results in a progeroid phenotype

Lamin A-dependent misregulation of adult stem cells associated with accelerated ageing

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

A lamin A protein isoform overexpressed in Hutchinson-Gilford progeria syndrome interferes with mitosis in progeria and normal cells

Alterations in mitosis and cell cycle progression caused by a mutant lamin A known to accelerate human aging

Prelamin A processing and heterochromatin dynamics in laminopathies

The mutant form of lamin A that causes Hutchinson-Gilford progeria is a biomarker of cellular aging in human skin

Increased progerin expression associated with unusual LMNA mutations causes severe progeroid syndromes

Aggrecan expression is substantially and abnormally upregulated in Hutchinson-Gilford Progeria Syndrome dermal fibroblasts

Hutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody

Rescue of heterochromatin organization in Hutchinson-Gilford progeria by drug treatment

Incomplete processing of mutant lamin A in Hutchinson-Gilford progeria leads to nuclear abnormalities, which are reversed by farnesyltransferase inhibition

Genomic instability in laminopathy-based premature aging

Novel progerin-interactive partner proteins hnRNP E1, EGF, Mel 18, and UBC9 interact with lamin A/C

Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndrome

Recurrent de novo point mutations in lamin A cause Hutchinson-Gilford progeria syndrome

First Generation Proteolysis Targeting Chimeras (PROTACs) for the Treatment of Progeria

Macicior-Michelena J, Telechea M, Fernández D, García-Martín A, Canales Á, Ortega-Gutiérrez S. Adv Sci (Weinh). Published online March 23, 2026. doi:10.1002/advs.202521608

2025

Lonafarnib Clinical Trials Demonstrate Uncoupling of the Muscle-Bone Unit in Hutchinson-Gilford Progeria Syndrome

Kreienkamp RJ, Gordon LB, Ehrbar

Baricitinib Augments Lonafarnib Therapy to Preserve Colonic Homeostasis and Microbial Balance in a Mouse Model of Progeria

Schroll M, Amar Y, Krüger P, Neuhaus K, Djabali K. Aging Cell. Published online October 20, 2025. doi:10.1111/acel.70273

Baricitinib and Lonafarnib Synergistically Target Progerin and Inflammation, Improving Lifespan and Health in Progeria Mice

Krüger P, Schroll M, Fenzl FQ, et al. Int J Mol Sci. 2025;26(10):4849. Published 2025 May 19. doi:10.3390/ijms26104849

Hutchinson-Gilford Progeria Syndrome

Gordon LB, Brown WT, Collins FS. 2003 Dec 12 [Updated 2025 Mar 13]. In: Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2025.

Restoring neuropetide Y levels in the hypothalamus ameliorates premature aging phenotype in mice

Ferreira-Marques M, Carmo-Silva S, Pereira J, et al. Geroscience. Published online February 27, 2025. doi:10.1007/s11357-025-01574-0

Longitudinal Changes in Myocardial Deformation in Hutchinson-Gilford Progeria Syndrome

Olsen FJ, Biering-Sørensen T, Lunze FI, et al. Circ Cardiovasc Imaging. 2025;18(2):e017544. doi:10.1161/CIRCIMAGING.124.017544

2024

Intervention for critical aortic stenosis in Hutchinson-Gilford progeria syndrome

Gordon LB, Basso S, Maestranzi J, et al. Front Cardiovasc Med. 2024;11:1356010. Published 2024 Apr 25. doi:10.3389/fcvm.2024.1356010

Inflammation and Fibrosis in Progeria: Organ-Specific Responses in an HGPS Mouse Model

Krüger P, Schroll M, Fenzl F, et al. Int J Mol Sci. 2024;25(17):9323. Published 2024 Aug 28. doi:10.3390/ijms25179323

The NLRP3 inhibitor Dapansutrile improves the therapeutic action of lonafarnib on progeroid mice

Muela-Zarzuela I, Suarez-Rivero JM, Boy-Ruiz D, et al. Aging Cell. 2024;23(9):e14272. doi:10.1111/acel.14272

Abnormal Myocardial Deformation Despite Normal Ejection Fraction in Hutchinson-Gilford Progeria Syndrome

Olsen FJ, Biering-Sørensen T, Lunze F, et al. J Am Heart Assoc. 2024;13(3):e031470. doi:10.1161/JAHA.123.031470

Vascular Calcification: A Passive Process That Requires Active Inhibition

Villa-Bellosta R. Biology (Basel). 2024;13(2):111. Published 2024 Feb 9. doi:10.3390/biology13020111

Peripheral artery disease and outcomes: how can we improve risk prediction?

Yanamandala M, Goudot G, Gerhard-Herman MD. Eur Heart J. 2024;45(19):1750-1752. doi:10.1093/eurheartj/ehae154

2023

Ghrelin delays premature aging in Hutchinson-Gilford progeria syndrome

Ferreira-Marques M, Carvalho A, Franco AC, et al. Aging Cell. 2023;22(12):e13983. doi:10.1111/acel.13983

Plasma Progerin in Patients With Hutchinson-Gilford Progeria Syndrome: Immunoassay Development and Clinical Evaluation

Gordon LB, Norris W, Hamren S, et al. Circulation. 2023;147(23):1734-1744. doi:10.1161/CIRCULATIONAHA.122.060002

Impact of Combined Baricitinib and FTI Treatment on Adipogenesis in Hutchinson-Gilford Progeria Syndrome and Other Lipodystrophic Laminopathies

Hartinger R, Lederer EM, Schena E, Lattanzi G, Djabali K. Cells. 2023;12(10):1350. Published 2023 May 9. doi:10.3390/cells12101350

Turnover and replication analysis by isotope labeling (TRAIL) reveals the influence of tissue context on protein and organelle lifetimes

Hasper J, Welle K, Hryhorenko J, Ghaemmaghami S, Buchwalter A. Mol Syst Biol. 2023;19(4):e11393. doi:10.15252/msb.202211393

Long lifetime and tissue-specific accumulation of lamin A/C in Hutchinson-Gilford progeria syndrome

Hasper J, Welle K, Swovick K, Hryhorenko J, Ghaemmaghami S, Buchwalter A. J Cell Biol. 2024;223(1):e202307049. doi:10.1083/jcb.202307049

Progerinin, an Inhibitor of Progerin, Alleviates Cardiac Abnormalities in a Model Mouse of Hutchinson-Gilford Progeria Syndrome

Kang SM, Seo S, Song EJ, et al. Cells. 2023;12(9):1232. Published 2023 Apr 24. doi:10.3390/cells12091232

A new fluorescent probe for the visualization of progerin

Macicior J, Fernández D, Ortega-Gutiérrez S. Bioorg Chem. 2024;142:106967. doi:10.1016/j.bioorg.2023.106967

Baseline Range of Motion, Strength, Motor Function, and Participation in Youth with Hutchinson-Gilford Progeria Syndrome

Malloy J, Berry E, Correia A, et al. Phys Occup Ther Pediatr. 2023;43(4):482-501. doi:10.1080/01942638.2022.2158054

The farnesyl transferase inhibitor (FTI) lonafarnib improves nuclear morphology in ZMPSTE24-deficient fibroblasts from patients with the progeroid disorder MAD-B

Odinammadu KO, Shilagardi K, Tuminelli K, Judge DP, Gordon LB, Michaelis S. Nucleus. 2023;14(1):2288476. doi:10.1080/19491034.2023.2288476

Progression of Cardiac Abnormalities in Hutchinson-Gilford Progeria Syndrome: A Prospective Longitudinal Study

Olsen FJ, Gordon LB, Smoot L, et al. Circulation. 2023;147(23):1782-1784. doi:10.1161/CIRCULATIONAHA.123.064370

2022

Progeria: a perspective on potential drug targets and treatment strategies

Benedicto I, Chen X, Bergo MO, Andrés V. Expert Opin Ther Targets. 2022;26(5):393-399. doi:10.1080/14728222.2022.2078699

Quantification of Farnesylated Progerin in Hutchinson-Gilford Progeria Patient Cells by Mass Spectrometry

Camafeita E, Jorge I, Rivera-Torres J, Andrés V, Vázquez J. Int J Mol Sci. 2022;23(19):11733. Published 2022 Oct 3. doi:10.3390/ijms231911733

Clonal hematopoiesis is not prevalent in Hutchinson-Gilford progeria syndrome

Díez-Díez M, Amorós-Pérez M, de la Barrera J, et al. Geroscience. 2023;45(2):1231-1236. doi:10.1007/s11357-022-00607-2

Endothelial and systemic upregulation of miR-34a-5p fine-tunes senescence in progeria

Manakanatas C, Ghadge SK, Agic A, et al. Aging (Albany NY). 2022;14(1):195-224. doi:10.18632/aging.203820

2021

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

Benedicto I, Dorado B, Andrés V. Cells. 2021;10(5):1157. Published 2021 May 11. doi:10.3390/cells10051157

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

Chen X, Yao H, Kashif M, et al. Elife. 2021;10:e63284. Published 2021 Feb 2. doi:10.7554/eLife.63284

A targeted antisense therapeutic approach for Hutchinson-Gilford progeria syndrome

Erdos MR, Cabral WA, Tavarez UL, et al. Nat Med. 2021;27(3):536-545. doi:10.1038/s41591-021-01274-0

Inhibition of the NLRP3 inflammasome improves lifespan in animal murine model of Hutchinson-Gilford Progeria

González-Dominguez A, Montañez R, Castejón-Vega B, et al. EMBO Mol Med. 2021;13(10):e14012. doi:10.15252/emmm.202114012

Progerinin, an optimized progerin-lamin A binding inhibitor, ameliorates premature senescence phenotypes of Hutchinson-Gilford progeria syndrome

Kang SM, Yoon MH, Ahn J, et al. [published correction appears in Commun Biol. 2021 Mar 2;4(1):297. doi: 10.1038/s42003-021-01843-6]. Commun Biol. 2021;4(1):5. Published 2021 Jan 4. doi:10.1038/s42003-020-01540-w

In vivo base editing rescues Hutchinson-Gilford progeria syndrome in mice

Koblan LW, Erdos MR, Wilson C, et al. Nature. 2021;589(7843):608-614. doi:10.1038/s41586-020-03086-7

A novel homozygous synonymous variant further expands the phenotypic spectrum of POLR3A-related pathologies

Lessel D, Rading K, Campbell SE, et al. Am J Med Genet A. 2022;188(1):216-223. doi:10.1002/ajmg.a.62525

Paclitaxel mitigates structural alterations and cardiac conduction system defects in a mouse model of Hutchinson-Gilford progeria syndrome

Macías Á, Díaz-Larrosa JJ, Blanco Y, et al. Cardiovasc Res. 2022;118(2):503-516. doi:10.1093/cvr/cvab055

Small-Molecule Therapeutic Perspectives for the Treatment of Progeria

Macicior J, Marcos-Ramiro B, Ortega-Gutiérrez S. Int J Mol Sci. 2021;22(13):7190. Published 2021 Jul 3. doi:10.3390/ijms22137190

Isoprenylcysteine Carboxylmethyltransferase-Based Therapy for Hutchinson-Gilford Progeria Syndrome

Marcos-Ramiro B, Gil-Ordóñez A, Marín-Ramos NI, et al. ACS Cent Sci. 2021;7(8):1300-1310. doi:10.1021/acscentsci.0c01698

Systematic screening identifies therapeutic antisense oligonucleotides for Hutchinson-Gilford progeria syndrome

Puttaraju M, Jackson M, Klein S, et al. [published correction appears in Nat Med. 2021 Jul;27(7):1309. doi: 10.1038/s41591-021-01415-5]. Nat Med. 2021;27(3):526-535. doi:10.1038/s41591-021-01262-4

Lifelong restriction of dietary branched-chain amino acids has sex-specific benefits for frailty and lifespan in mice

Richardson NE, Konon EN, Schuster HS, et al. Nat Aging. 2021;1(1):73-86. doi:10.1038/s43587-020-00006-2

Interleukin-6 neutralization ameliorates symptoms in prematurely aged mice

Squarzoni S, Schena E, Sabatelli P, et al. Aging Cell. 2021;20(1):e13285. doi:10.1111/acel.13285

Decreased vascular smooth muscle contractility in Hutchinson-Gilford Progeria Syndrome linked to defective smooth muscle myosin heavy chain expression

von Kleeck R, Castagnino P, Roberts E, Talwar S, Ferrari G, Assoian RK. Sci Rep. 2021;11(1):10625. Published 2021 May 19. doi:10.1038/s41598-021-90119-4

2020

Neuropeptide Y Enhances Progerin Clearance and Ameliorates the Senescent Phenotype of Human Hutchinson-Gilford Progeria Syndrome Cells

Aveleira CA, Ferreira-Marques M, Cortes L, et al. J Gerontol A Biol Sci Med Sci. 2020;75(6):1073-1078. doi:10.1093/gerona/glz280

Interplay of the nuclear envelope with chromatin in physiology and pathology

Burla R, La Torre M, Maccaroni K, Verni F, Giunta S, Saggio I. Nucleus. 2020;11(1):205-218. doi:10.1080/19491034.2020.1806661

Lamin A involvement in ageing processes

Cenni V, Capanni C, Mattioli E, et al. Ageing Res Rev. 2020;62:101073. doi:10.1016/j.arr.2020.101073

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

Cubria MB, Suarez S, Masoudi A, et al. Proc Natl Acad Sci U S A. 2020;117(22):12029-12040. doi:10.1073/pnas.1906713117

Identification of common cardiometabolic alterations and deregulated pathways in mouse and pig models of aging

Fanjul V, Jorge I, Camafeita E, et al. Aging Cell. 2020;19(9):e13203. doi:10.1111/acel.13203

Phosphorylated Lamin A/C in the Nuclear Interior Binds Active Enhancers Associated with Abnormal Transcription in Progeria

Ikegami K, Secchia S, Almakki O, Lieb JD, Moskowitz IP. Dev Cell. 2020;52(6):699-713.e11. doi:10.1016/j.devcel.2020.02.011

Cytoskeleton stiffness regulates cellular senescence and innate immune response in Hutchinson-Gilford Progeria Syndrome

Mu X, Tseng C, Hambright WS, et al. Aging Cell. 2020;19(8):e13152. doi:10.1111/acel.13152

First progeria monkey model generated using base editor

Reddy P, Shao Y, Hernandez-Benitez R, Nuñez Delicado E, Izpisua Belmonte JC. Protein Cell. 2020;11(12):862-865. doi:10.1007/s13238-020-00765-z

Skeletal maturation and long-bone growth patterns of patients with progeria: a retrospective study

Tsai A, Johnston PR, Gordon LB, Walters M, Kleinman M, Laor T. Lancet Child Adolesc Health. 2020;4(4):281-289. doi:10.1016/S2352-4642(20)30023-7

New treatments for progeria

Villa-Bellosta R. Aging (Albany NY). 2019;11(24):11801-11802. doi:10.18632/aging.102626

Dietary magnesium supplementation improves lifespan in a mouse model of progeria

Villa-Bellosta R. EMBO Mol Med. 2020;12(10):e12423. doi:10.15252/emmm.202012423

Redox theory in progeria

Villa-Bellosta R. Aging (Albany NY). 2020;12(21):20934-20935. doi:10.18632/aging.104211

2019

Healthspan and lifespan extension by fecal microbiota transplantation into progeroid mice

Bárcena C, Valdés-Mas R, Mayoral P, et al. Nat Med. 2019;25(8):1234-1242. doi:10.1038/s41591-019-0504-5

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

Beyret E, Liao HK, Yamamoto M, et al. Nat Med. 2019;25(3):419-422. doi:10.1038/s41591-019-0343-4

Generation and characterization of a novel knockin minipig model of Hutchinson-Gilford progeria syndrome

Dorado B, Pløen GG, Barettino A, et al. Cell Discov. 2019;5:16. Published 2019 Mar 19. doi:10.1038/s41421-019-0084-z

Extraskeletal Calcifications in Hutchinson-Gilford Progeria Syndrome

Gordon CM, Cleveland RH, Baltrusaitis K, et al. Bone. 2019;125:103-111. doi:10.1016/j.bone.2019.05.008

Vascular smooth muscle cell loss underpins the accelerated atherosclerosis in Hutchinson-Gilford progeria syndrome

Hamczyk MR, Andrés V. Nucleus. 2019;10(1):28-34. doi:10.1080/19491034.2019.1589359

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

Hamczyk MR, Villa-Bellosta R, Quesada V, et al. EMBO Mol Med. 2019;11(4):e9736. doi:10.15252/emmm.201809736

Remodeling of Bone Marrow Hematopoietic Stem Cell Niches Promotes Myeloid Cell Expansion during Premature or Physiological Aging

Ho YH, Del Toro R, Rivera-Torres J, et al. Cell Stem Cell. 2019;25(3):407-418.e6. doi:10.1016/j.stem.2019.06.007

Dysfunction of iPSC-derived endothelial cells in human Hutchinson-Gilford progeria syndrome

Matrone G, Thandavarayan RA, Walther BK, Meng S, Mojiri A, Cooke JP. Cell Cycle. 2019;18(19):2495-2508. doi:10.1080/15384101.2019.1651587

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

Santiago-Fernández O, Osorio FG, Quesada V, et al. Nat Med. 2019;25(3):423-426. doi:10.1038/s41591-018-0338-6

Physicochemical mechanotransduction alters nuclear shape and mechanics via heterochromatin formation

Stephens AD, Liu PZ, Kandula V, et al. Mol Biol Cell. 2019;30(17):2320-2330. doi:10.1091/mbc.E19-05-0286

ATP-based therapy prevents vascular calcification and extends longevity in a mouse model of Hutchinson-Gilford progeria syndrome

Villa-Bellosta R. Proc Natl Acad Sci U S A. 2019;116(47):23698-23704. doi:10.1073/pnas.1910972116

Impact of acetate- or citrate-acidified bicarbonate dialysate on ex vivo aorta wall calcification

Villa-Bellosta R, Hernández-Martínez E, Mérida-Herrero E, González-Parra E. Sci Rep. 2019;9(1):11374. Published 2019 Aug 6. doi:10.1038/s41598-019-47934-7

Questioning the Safety of Calcidiol in Hemodialysis Patients

Villa-Bellosta R, Mahillo-Fernández I, Ortíz A, González-Parra E. Nutrients. 2019;11(5):959. Published 2019 Apr 26. doi:10.3390/nu11050959

2018

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

Bárcena C, Quirós PM, Durand S, et al. Cell Rep. 2018;24(9):2392-2403. doi:10.1016/j.celrep.2018.07.089

Microbiome at sites of gingival recession in children with Hutchinson-Gilford progeria syndrome

Bassir SH, Chase I, Paster BJ, et al. J Periodontol. 2018;89(6):635-644. doi:10.1002/JPER.17-0351

Genomic instability and DNA replication defects in progeroid syndromes

Burla R, La Torre M, Merigliano C, Vernì F, Saggio I. Nucleus. 2018;9(1):368-379. doi:10.1080/19491034.2018.1476793

Mouse Models to Disentangle the Hallmarks of Human Aging

Folgueras AR, Freitas-Rodríguez S, Velasco G, López-Otín C. Circ Res. 2018;123(7):905-924. doi:10.1161/CIRCRESAHA.118.312204

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

Gordon LB, Campbell SE, Massaro JM, et al. Pediatr Res. 2018;83(5):982-992. doi:10.1038/pr.2018.9

Association of Lonafarnib Treatment vs No Treatment With Mortality Rate in Patients With Hutchinson-Gilford Progeria Syndrome

Gordon LB, Shappell H, Massaro J, et al. JAMA. 2018;319(16):1687-1695. doi:10.1001/jama.2018.3264

Pubertal Progression in Female Adolescents with Progeria

Greer MM, Kleinman ME, Gordon LB, et al. J Pediatr Adolesc Gynecol. 2018;31(3):238-241. doi:10.1016/j.jpag.2017.12.005

Accelerated atherosclerosis in HGPS

Hamczyk MR, Andrés V. Aging (Albany NY). 2018;10(10):2555-2556. doi:10.18632/aging.101608

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

Hamczyk MR, Villa-Bellosta R, Gonzalo P, et al. Circulation. 2018;138(3):266-282. doi:10.1161/CIRCULATIONAHA.117.030856

Analyses of LMNA-negative juvenile progeroid cases confirms biallelic POLR3A mutations in Wiedemann-Rautenstrauch-like syndrome and expands the phenotypic spectrum of PYCR1 mutations

Lessel D, Ozel AB, Campbell SE, et al. Hum Genet. 2018;137(11-12):921-939. doi:10.1007/s00439-018-1957-1

Endothelial progerin expression causes cardiovascular pathology through an impaired mechanoresponse

Osmanagic-Myers S, Kiss A, Manakanatas C, et al. J Clin Invest. 2019;129(2):531-545. doi:10.1172/JCI121297

Cardiac Abnormalities in Patients With Hutchinson-Gilford Progeria Syndrome

Prakash A, Gordon LB, Kleinman ME, et al. JAMA Cardiol. 2018;3(4):326-334. doi:10.1001/jamacardio.2017.5235

OGT (O-GlcNAc Transferase) Selectively Modifies Multiple Residues Unique to Lamin A

Simon DN, Wriston A, Fan Q, et al. Cells. 2018;7(5):44. Published 2018 May 17. doi:10.3390/cells7050044

Nuclear import pathway key to rescuing dominant progerin phenotypes

Wilson KL. Sci Signal. 2018;11(537):eaat9448. Published 2018 Jul 3. doi:10.1126/scisignal.aat9448

Differential stem cell aging kinetics in Hutchinson-Gilford progeria syndrome and Werner syndrome

Wu Z, Zhang W, Song M, et al. Protein Cell. 2018;9(4):333-350. doi:10.1007/s13238-018-0517-8

2017

Functional relevance of miRNAs in premature ageing

Caravia XM, Roiz-Valle D, Morán-Álvarez A, López-Otín C. Mech Ageing Dev. 2017;168:10-19. doi:10.1016/j.mad.2017.05.003

Reprogramming progeria fibroblasts re-establishes a normal epigenetic landscape

Chen Z, Chang WY, Etheridge A, et al. Aging Cell. 2017;16(4):870-887. doi:10.1111/acel.12621

A-type lamins and cardiovascular disease in premature aging syndromes

Dorado B, Andrés V. Curr Opin Cell Biol. 2017;46:17-25. doi:10.1016/j.ceb.2016.12.005

LMNA Sequences of 60,706 Unrelated Individuals Reveal 132 Novel Missense Variants in A-Type Lamins and Suggest a Link between Variant p.G602S and Type 2 Diabetes – PubMed (nih.gov)

Florwick A, Dharmaraj T, Jurgens J, Valle D, Wilson KL.  Front Genet. 2017;8:79. Published 2017 Jun 15. doi:10.3389/fgene.2017.00079

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

Gabriel D, Shafry DD, Gordon LB, Djabali K. Oncotarget. 2017;8(39):64809-64826. Published 2017 Jul 18. doi:10.18632/oncotarget.19363

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

Hamczyk MR, del Campo L, Andrés V. Annu Rev Physiol. 2018;80:27-48. doi:10.1146/annurev-physiol-021317-121454

Progerin sequestration of PCNA promotes replication fork collapse and mislocalization of XPA in laminopathy-related progeroid syndromes

Hilton BA, Liu J, Cartwright BM, et al. FASEB J. 2017;31(9):3882-3893. doi:10.1096/fj.201700014R

Mice with reduced expression of the telomere-associated protein Ft1 develop p53-sensitive progeroid traits

La Torre M, Merigliano C, Burla R, et al. Aging Cell. 2018;17(4):e12730. doi:10.1111/acel.12730

Telomerase mRNA Reverses Senescence in Progeria Cells

Li Y, Zhou G, Bruno IG, Cooke JP. J Am Coll Cardiol. 2017;70(6):804-805. doi:10.1016/j.jacc.2017.06.017

Ophthalmologic Features of Progeria

Mantagos IS, Kleinman ME, Kieran MW, Gordon LB. Am J Ophthalmol. 2017;182:126-132. doi:10.1016/j.ajo.2017.07.020

Protein sequestration at the nuclear periphery as a potential regulatory mechanism in premature aging

Serebryannyy L, Misteli T. J Cell Biol. 2018;217(1):21-37. doi:10.1083/jcb.201706061

Chromatin and lamin A determine two different mechanical response regimes of the cell nucleus

Stephens AD, Banigan EJ, Adam SA, Goldman RD, Marko JF. Mol Biol Cell. 2017;28(14):1984-1996. doi:10.1091/mbc.E16-09-0653

Chromatin histone modifications and rigidity affect nuclear morphology independent of lamins

Stephens AD, Liu PZ, Banigan EJ, et al. Mol Biol Cell. 2018;29(2):220-233. doi:10.1091/mbc.E17-06-0410

The molecular architecture of lamins in somatic cells

Turgay Y, Eibauer M, Goldman AE, et al. Nature. 2017;543(7644):261-264. doi:10.1038/nature21382

Nucleoplasmic lamins define growth-regulating functions of lamina-associated polypeptide 2α in progeria cells

Vidak S, Georgiou K, Fichtinger P, Naetar N, Dechat T, Foisner R. J Cell Sci. 2018;131(3):jcs208462. Published 2018 Feb 8. doi:10.1242/jcs.208462

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

Wheaton K, Campuzano D, Ma W, et al. Mol Cell Biol. 2017;37(14):e00659-16. Published 2017 Jun 29. doi:10.1128/MCB.00659-16

Substrate stiffness-dependent regulation of the SRF-Mkl1 co-activator complex requires the inner nuclear membrane protein Emerin

Willer MK, Carroll CW. J Cell Sci. 2017;130(13):2111-2118. doi:10.1242/jcs.197517

2016

A novel somatic mutation achieves partial rescue in a child with Hutchinson-Gilford progeria syndrome

Bar DZ, Arlt MF, Brazier JF, et al. J Med Genet. 2017;54(3):212-216. doi:10.1136/jmedgenet-2016-104295

Simple Separation of Functionally Distinct Populations of Lamin-Binding Proteins

Berk JM, Wilson KL. Methods Enzymol. 2016;569:101-114. doi:10.1016/bs.mie.2015.09.034

Seeking a Cure for One of the Rarest Diseases: Progeria

Collins FS. Circulation. 2016;134(2):126-129. doi:10.1161/CIRCULATIONAHA.116.022965

Progerin impairs chromosome maintenance by depleting CENP-F from metaphase kinetochores in Hutchinson-Gilford progeria fibroblasts

Eisch V, Lu X, Gabriel D, Djabali K. Oncotarget. 2016;7(17):24700-24718. doi:10.18632/oncotarget.8267

Temsirolimus Partially Rescues the Hutchinson-Gilford Progeria Cellular Phenotype

Gabriel D, Gordon LB, Djabali K. PLoS One. 2016;11(12):e0168988. Published 2016 Dec 29. doi:10.1371/journal.pone.0168988

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

Gordon LB, Kleinman ME, Massaro J, et al. Circulation. 2016;134(2):114-125. doi:10.1161/CIRCULATIONAHA.116.022188

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

Kreienkamp R, Croke M, Neumann MA, et al. Oncotarget. 2016;7(21):30018-30031. doi:10.18632/oncotarget.9065

Repression of the Antioxidant NRF2 Pathway in Premature Aging

Kubben N, Zhang W, Wang L, et al. Cell. 2016;165(6):1361-1374. doi:10.1016/j.cell.2016.05.017

Interruption of progerin-lamin A/C binding ameliorates Hutchinson-Gilford progeria syndrome phenotype

Lee SJ, Jung YS, Yoon MH, et al. J Clin Invest. 2016;126(10):3879-3893. doi:10.1172/JCI84164

Permanent farnesylation of lamin A mutants linked to progeria impairs its phosphorylation at serine 22 during interphase

Moiseeva O, Lopes-Paciencia S, Huot G, Lessard F, Ferbeyre G. Aging (Albany NY). 2016;8(2):366-381. doi:10.18632/aging.100903

Cardiac electrical defects in progeroid mice and Hutchinson-Gilford progeria syndrome patients with nuclear lamina alterations

Rivera-Torres J, Calvo CJ, Llach A, et al. Proc Natl Acad Sci U S A. 2016;113(46):E7250-E7259. doi:10.1073/pnas.1603754113

Molecular insights into the premature aging disease progeria

Vidak S, Foisner R. Histochem Cell Biol. 2016;145(4):401-417. doi:10.1007/s00418-016-1411-1

2015

ADAMTS7 in cardiovascular disease: from bedside to bench and back again?

Arroyo AG, Andrés V. Circulation. 2015;131(13):1156-1159. doi:10.1161/CIRCULATIONAHA.115.015711

Progerin reduces LAP2α-telomere association in Hutchinson-Gilford progeria

Chojnowski A, Ong PF, Wong ES, et al. Elife. 2015;4:e07759. Published 2015 Aug 27. doi:10.7554/eLife.07759

Autophagy mediates degradation of nuclear lamina

Dou Z, Xu C, Donahue G, et al. Nature. 2015;527(7576):105-109. doi:10.1038/nature15548

The tail domain of lamin B1 is more strongly modulated by divalent cations than lamin A

Ganesh S, Qin Z, Spagnol ST, et al. Nucleus. 2015;6(3):203-211. doi:10.1080/19491034.2015.1031436

A high-content imaging-based screening pipeline for the systematic identification of anti-progeroid compounds

Kubben N, Brimacombe KR, Donegan M, Li Z, Misteli T. Methods. 2016;96:46-58. doi:10.1016/j.ymeth.2015.08.024

Mutant lamin A links prophase to a p53 independent senescence program

Moiseeva O, Lessard F, Acevedo-Aquino M, Vernier M, Tsantrizos YS, Ferbeyre G. Cell Cycle. 2015;14(15):2408-2421. doi:10.1080/15384101.2015.1053671

Lamins at the crossroads of mechanosignaling

Osmanagic-Myers S, Dechat T, Foisner R. Genes Dev. 2015;29(3):225-237. doi:10.1101/gad.255968.114

Gene-rich chromosomal regions are preferentially localized in the lamin B deficient nuclear blebs of atypical progeria cells

Bercht Pfleghaar K, Taimen P, Butin-Israeli V, et al. [published correction appears in Nucleus. 2015;6(3):247. doi: 10.1080/19491034.2015.1049921]. Nucleus. 2015;6(1):66-76. doi:10.1080/19491034.2015.1004256

Structural organization of nuclear lamins A, C, B1, and B2 revealed by superresolution microscopy

Shimi T, Kittisopikul M, Tran J, et al. Mol Biol Cell. 2015;26(22):4075-4086. doi:10.1091/mbc.E15-07-0461

Transgene silencing of the Hutchinson-Gilford progeria syndrome mutation results in a reversible bone phenotype, whereas resveratrol treatment does not show overall beneficial effects

Strandgren C, Nasser HA, McKenna T, et al. FASEB J. 2015;29(8):3193-3205. doi:10.1096/fj.14-269217

Proliferation of progeria cells is enhanced by lamina-associated polypeptide 2α (LAP2α) through expression of extracellular matrix proteins

Vidak S, Kubben N, Dechat T, Foisner R. Genes Dev. 2015;29(19):2022-2036. doi:10.1101/gad.263939.115

2014

Expression of progerin in aging mouse brains reveals structural nuclear abnormalities without detectible significant alterations in gene expression, hippocampal stem cells or behavior

Baek JH, Schmidt E, Viceconte N, et al. Hum Mol Genet. 2015;24(5):1305-1321. doi:10.1093/hmg/ddu541

The non-random repositioning of whole chromosomes and individual gene loci in interphase nuclei and its relevance in disease, infection, aging, and cancer

Bridger JM, Arican-Gotkas HD, Foster HA, et al. [published correction appears in Adv Exp Med Biol. 2014;773:E1]. Adv Exp Med Biol. 2014;773:263-279. doi:10.1007/978-1-4899-8032-8_12

Role of lamin b1 in chromatin instability

Butin-Israeli V, Adam SA, Jain N, et al.. Mol Cell Biol. 2015;35(5):884-898. doi:10.1128/MCB.01145-14

Systematic identification of pathological lamin A interactors

Dittmer TA, Sahni N, Kubben N, et al. Mol Biol Cell. 2014;25(9):1493-1510. doi:10.1091/mbc.E14-02-0733

Sulforaphane enhances progerin clearance in Hutchinson-Gilford progeria fibroblasts

Gabriel D, Roedl D, Gordon LB, Djabali K. Aging Cell. 2015;14(1):78-91. doi:10.1111/acel.12300

Sorting nexin 6 enhances lamin a synthesis and incorporation into the nuclear envelope

González-Granado JM, Navarro-Puche A, Molina-Sanchez P, et al. PLoS One. 2014;9(12):e115571. Published 2014 Dec 23. doi:10.1371/journal.pone.0115571

Nuclear envelope lamin-A couples actin dynamics with immunological synapse architecture and T cell activation

González-Granado JM, Silvestre-Roig C, Rocha-Perugini V, et al. Sci Signal. 2014;7(322):ra37. Published 2014 Apr 22. doi:10.1126/scisignal.2004872

Interfacial binding and aggregation of lamin A tail domains associated with Hutchinson-Gilford progeria syndrome

Kalinowski A, Yaron PN, Qin Z, et al. Biophys Chem. 2014;195:43-48. doi:10.1016/j.bpc.2014.08.005

Interphase phosphorylation of lamin A

Kochin V, Shimi T, Torvaldson E, et al. J Cell Sci. 2014;127(Pt 12):2683-2696. doi:10.1242/jcs.141820

Mouse models and aging: longevity and progeria

Liao CY, Kennedy BK. Curr Top Dev Biol. 2014;109:249-285. doi:10.1016/B978-0-12-397920-9.00003-2

Atherosclerosis in ancient humans, accelerated aging syndromes and normal aging: is lamin a protein a common link?

Miyamoto MI, Djabali K, Gordon LB. Glob Heart. 2014;9(2):211-218. doi:10.1016/j.gheart.2014.04.001

Initial cutaneous manifestations of Hutchinson-Gilford progeria syndrome

Rork JF, Huang JT, Gordon LB, Kleinman M, Kieran MW, Liang MG. Pediatr Dermatol. 2014;31(2):196-202. doi:10.1111/pde.12284

2013

Regulation of nucleotide excision repair by nuclear lamin b1

Butin-Israeli V, Adam SA, Goldman RD. PLoS One. 2013;8(7):e69169. Published 2013 Jul 24. doi:10.1371/journal.pone.0069169

Broken nuclei–lamins, nuclear mechanics, and disease

Davidson PM, Lammerding J. Trends Cell Biol. 2014;24(4):247-256. doi:10.1016/j.tcb.2013.11.004

Mechanical model of blebbing in nuclear lamin meshworks

Funkhouser CM, Sknepnek R, Shimi T, Goldman AE, Goldman RD, Olvera de la Cruz M. Proc Natl Acad Sci U S A. 2013;110(9):3248-3253. doi:10.1073/pnas.1300215110

Lamin A/C and emerin regulate MKL1-SRF activity by modulating actin dynamics

Ho CY, Jaalouk DE, Vartiainen MK, Lammerding J. Nature. 2013;497(7450):507-511. doi:10.1038/nature12105

Nuclear mechanics and mechanotransduction in health and disease

Isermann P, Lammerding J. Curr Biol. 2013;23(24):R1113-R1121. doi:10.1016/j.cub.2013.11.009

Calcium causes a conformational change in lamin A tail domain that promotes farnesyl-mediated membrane association

Kalinowski A, Qin Z, Coffey K, et al. Biophys J. 2013;104(10):2246-2253. doi:10.1016/j.bpj.2013.04.016

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

Rivera-Torres J, Acín-Perez R, Cabezas-Sánchez P, et al. J Proteomics. 2013;91:466-477. doi:10.1016/j.jprot.2013.08.008

Imaging characteristics of cerebrovascular arteriopathy and stroke in Hutchinson-Gilford progeria syndrome

Silvera VM, Gordon LB, Orbach DB, Campbell SE, Machan JT, Ullrich NJ. AJNR Am J Neuroradiol. 2013;34(5):1091-1097. doi:10.3174/ajnr.A3341

Neurologic features of Hutchinson-Gilford progeria syndrome after lonafarnib treatment

Ullrich NJ, Kieran MW, Miller DT, et al. Neurology. 2013;81(5):427-430. doi:10.1212/WNL.0b013e31829d85c0

Defective extracellular pyrophosphate metabolism promotes vascular calcification in a mouse model of Hutchinson-Gilford progeria syndrome that is ameliorated on pyrophosphate treatment

Villa-Bellosta R, Rivera-Torres J, Osorio FG, et al. Circulation. 2013;127(24):2442-2451. doi:10.1161/CIRCULATIONAHA.112.000571

2012

A prospective study of radiographic manifestations in Hutchinson-Gilford progeria syndrome

Cleveland RH, Gordon LB, Kleinman ME, et al. Pediatr Radiol. 2012;42(9):1089-1098. doi:10.1007/s00247-012-2423-1

Automated image analysis of nuclear shape: what can we learn from a prematurely aged cell?

Driscoll MK, Albanese JL, Xiong ZM, Mailman M, Losert W, Cao K. Aging (Albany NY). 2012;4(2):119-132. doi:10.18632/aging.100434

Mechanisms of premature vascular aging in children with Hutchinson-Gilford progeria syndrome

Gerhard-Herman M, Smoot LB, Wake N, et al. Hypertension. 2012;59(1):92-97. doi:10.1161/HYPERTENSIONAHA.111.180919

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

Gordon LB, Kleinman ME, Miller DT, et al. Proc Natl Acad Sci U S A. 2012;109(41):16666-16671. doi:10.1073/pnas.1202529109

Progeria: translational insights from cell biology

Gordon LB, Cao K, Collins FS. J Cell Biol. 2012;199(1):9-13. doi:10.1083/jcb.201207072

Resveratrol rescues SIRT1-dependent adult stem cell decline and alleviates progeroid features in laminopathy-based progeria

Liu B, Ghosh S, Yang X, et al. Cell Metab. 2012;16(6):738-750. doi:10.1016/j.cmet.2012.11.007

Replication factor C1, the large subunit of replication factor C, is proteolytically truncated in Hutchinson-Gilford progeria syndrome

Tang H, Hilton B, Musich PR, Fang DZ, Zou Y. Aging Cell. 2012;11(2):363-365. doi:10.1111/j.1474-9726.2011.00779.x

Craniofacial abnormalities in Hutchinson-Gilford progeria syndrome

Ullrich NJ, Silvera VM, Campbell

2011

Accumulation of distinct prelamin A variants in human diploid fibroblasts differentially affects cell homeostasis

Candelario J, Borrego S, Reddy S, Comai L. Exp Cell Res. 2011;317(3):319-329. doi:10.1016/j.yexcr.2010.10.014

Computational image analysis of nuclear morphology associated with various nuclear-specific aging disorders

Choi S, Wang W, Ribeiro AJ, et al. Nucleus. 2011;2(6):570-579. doi:10.4161/nucl.2.6.17798

Hutchinson-Gilford progeria is a skeletal dysplasia

Gordon CM, Gordon LB, Snyder BD, et al.. J Bone Miner Res. 2011;26(7):1670-1679. doi:10.1002/jbmr.392

Age-dependent loss of MMP-3 in Hutchinson-Gilford progeria syndrome

Harten IA, Zahr RS, Lemire JM, et al. J Gerontol A Biol Sci Med Sci. 2011;66(11):1201-1207. doi:10.1093/gerona/glr137

The defective nuclear lamina in Hutchinson-gilford progeria syndrome disrupts the nucleocytoplasmic Ran gradient and inhibits nuclear localization of Ubc9

Kelley JB, Datta S, Snow CJ, et al. Mol Cell Biol. 2011;31(16):3378-3395. doi:10.1128/MCB.05087-11

‘Relax and Repair’ to restrain aging

Krishnan V, Liu B, Zhou Z. Aging (Albany NY). 2011;3(10):943-954. doi:10.18632/aging.100399

Histone H4 lysine 16 hypoacetylation is associated with defective DNA repair and premature senescence in Zmpste24-deficient mice

Krishnan V, Chow MZ, Wang Z, et al. Proc Natl Acad Sci U S A. 2011;108(30):12325-12330. doi:10.1073/pnas.1102789108

DNA-damage accumulation and replicative arrest in Hutchinson-Gilford progeria syndrome

Musich PR, Zou Y. Biochem Soc Trans. 2011;39(6):1764-1769. doi:10.1042/BST20110687

Structure and stability of the lamin A tail domain and HGPS mutant

Qin Z, Kalinowski A, Dahl KN, Buehler MJ. J Struct Biol. 2011;175(3):425-433. doi:10.1016/j.jsb.2011.05.015

Protein farnesylation inhibitors cause donut-shaped cell nuclei attributable to a centrosome separation defect

Verstraeten VL, Peckham LA, Olive M, et al. Proc Natl Acad Sci U S A. 2011;108(12):4997-5002. doi:10.1073/pnas.1019532108

2010

Mechanobiology and the microcirculation: cellular, nuclear and fluid mechanics

Dahl KN, Kalinowski A, Pekkan K. Microcirculation. 2010;17(3):179-191. doi:10.1111/j.1549-8719.2009.00016.x

Cardiovascular pathology in Hutchinson-Gilford progeria: correlation with the vascular pathology of aging

Olive M, Harten I, Mitchell R, et al. Arterioscler Thromb Vasc Biol. 2010;30(11):2301-2309. doi:10.1161/ATVBAHA.110.209460

2009

Association of progerin-interactive partner proteins with lamina proteins: Mel18 is associated with emerin in HGPS

Ju WN, Brown WT, Zhong N. Beijing Da Xue Xue Bao Yi Xue Ban. 2009;41(4):397-401.

Altered nuclear functions in progeroid syndromes: a paradigm for aging research

Li B, Jog S, Candelario J, Reddy S, Comai L. ScientificWorldJournal. 2009;9:1449-1462. Published 2009 Dec 16. doi:10.1100/tsw.2009.159

2008

Perturbation of wild-type lamin A metabolism results in a progeroid phenotype

Candelario J, Sudhakar S, Navarro S, Reddy S, Comai L. Aging Cell. 2008;7(3):355-367. doi:10.1111/j.1474-9726.2008.00393.x

Involvement of xeroderma pigmentosum group A (XPA) in progeria arising from defective maturation of prelamin A

Liu Y, Wang Y, Rusinol AE, et al. FASEB J. 2008;22(2):603-611. doi:10.1096/fj.07-8598com

Towards an integrated understanding of the structure and mechanics of the cell nucleus

Rowat AC, Lammerding J, Herrmann H, Aebi U. Bioessays. 2008;30(3):226-236. doi:10.1002/bies.20720

Lamin A-dependent misregulation of adult stem cells associated with accelerated ageing

Scaffidi P, Misteli T. Nat Cell Biol. 2008;10(4):452-459. doi:10.1038/ncb1708

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

Verstraeten VL, Ji JY, Cummings KS, Lee RT, Lammerding J. Aging Cell. 2008;7(3):383-393. doi:10.1111/j.1474-9726.2008.00382.x

Eliminating the synthesis of mature lamin A reduces disease phenotypes in mice carrying a Hutchinson-Gilford progeria syndrome allele

Yang SH, Qiao X, Farber E, Chang SY, Fong LG, Young SG. J Biol Chem. 2008;283(11):7094-7099. doi:10.1074/jbc.M708138200

Treatment with a farnesyltransferase inhibitor improves survival in mice with a Hutchinson-Gilford progeria syndrome mutation

Yang SH, Qiao X, Fong LG, Young SG. Biochim Biophys Acta. 2008;1781(1-2):36-39. doi:10.1016/j.bbalip.2007.11.003

2007

Disease progression in Hutchinson-Gilford progeria syndrome: impact on growth and development

Gordon LB, McCarten KM, Giobbie-Hurder A, et al. Pediatrics. 2007;120(4):824-833. doi:10.1542/peds.2007-1357

Cell nuclei spin in the absence of lamin b1

Ji JY, Lee RT, Vergnes L, et al. J Biol Chem. 2007;282(27):20015-20026. doi:10.1074/jbc.M611094200

The mutant form of lamin A that causes Hutchinson-Gilford progeria is a biomarker of cellular aging in human skin

McClintock D, Ratner D, Lokuge M, et al. PLoS One. 2007;2(12):e1269. Published 2007 Dec 5. doi:10.1371/journal.pone.0001269

Increased progerin expression associated with unusual LMNA mutations causes severe progeroid syndromes

Moulson CL, Fong LG, Gardner JM, et al. Hum Mutat. 2007;28(9):882-889. doi:10.1002/humu.20536

2006

Prelamin A and lamin A appear to be dispensable in the nuclear lamina

Fong LG, Ng JK, Lammerding J, et al. J Clin Invest. 2006;116(3):743-752. doi:10.1172/JCI27125

Aggrecan expression is substantially and abnormally upregulated in Hutchinson-Gilford Progeria Syndrome dermal fibroblasts

Lemire JM, Patis C, Gordon LB, Sandy JD, Toole BP, Weiss AS. Mech Ageing Dev. 2006;127(8):660-669. doi:10.1016/j.mad.2006.03.004

Nuclear lamins, diseases and aging

Mattout A, Dechat T, Adam SA, Goldman RD, Gruenbaum Y. Curr Opin Cell Biol. 2006;18(3):335-341. doi:10.1016/j.ceb.2006.03.007

Hutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody

McClintock D, Gordon LB, Djabali K. Proc Natl Acad Sci U S A. 2006;103(7):2154-2159. doi:10.1073/pnas.0511133103

Protein farnesyltransferase inhibitors and progeria

Meta M, Yang SH, Bergo MO, Fong LG, Young SG. Trends Mol Med. 2006;12(10):480-487. doi:10.1016/j.molmed.2006.08.006

Mutant nuclear lamin A leads to progressive alterations of epigenetic control in premature aging

Shumaker DK, Dechat T, Kohlmaier A, et al. Proc Natl Acad Sci U S A. 2006;103(23):8703-8708. doi:10.1073/pnas.0602569103

Prelamin A farnesylation and progeroid syndromes

Young SG, Meta M, Yang SH, Fong LG. J Biol Chem. 2006;281(52):39741-39745. doi:10.1074/jbc.R600033200

2005

Incomplete processing of mutant lamin A in Hutchinson-Gilford progeria leads to nuclear abnormalities, which are reversed by farnesyltransferase inhibition

Glynn MW, Glover TW. Hum Mol Genet. 2005;14(20):2959-2969. doi:10.1093/hmg/ddi326

Reduced adiponectin and HDL cholesterol without elevated C-reactive protein: clues to the biology of premature atherosclerosis in Hutchinson-Gilford Progeria Syndrome

Gordon LB, Harten IA, Patti ME, Lichtenstein AH. J Pediatr. 2005;146(3):336-341. doi:10.1016/j.jpeds.2004.10.064

Correction of cellular phenotypes of Hutchinson-Gilford Progeria cells by RNA interference

Huang S, Chen L, Libina N, et al. Hum Genet. 2005;118(3-4):444-450. doi:10.1007/s00439-005-0051-7

Inhibiting farnesylation reverses the nuclear morphology defect in a HeLa cell model for Hutchinson-Gilford progeria syndrome

Mallampalli MP, Huyer G, Bendale P, Gelb MH, Michaelis S. Proc Natl Acad Sci U S A. 2005;102(40):14416-14421. doi:10.1073/pnas.0503712102

Dermal fibroblasts in Hutchinson-Gilford progeria syndrome with the lamin A G608G mutation have dysmorphic nuclei and are hypersensitive to heat stress

Paradisi M, McClintock D, Boguslavsky RL, Pedicelli C, Worman HJ, Djabali K. BMC Cell Biol. 2005;6:27. Published 2005 Jun 27. doi:10.1186/1471-2121-6-27

Blocking protein farnesyltransferase improves nuclear shape in fibroblasts from humans with progeroid syndromes

Toth JI, Yang SH, Qiao X, et al. Proc Natl Acad Sci U S A. 2005;102(36):12873-12878. doi:10.1073/pnas.0505767102

Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts with a targeted Hutchinson-Gilford progeria syndrome mutation

Yang SH, Bergo MO, Toth JI, et al. Proc Natl Acad Sci U S A. 2005;102(29):10291-10296. doi:10.1073/pnas.0504641102

Prelamin A, Zmpste24, misshapen cell nuclei, and progeria–new evidence suggesting that protein farnesylation could be important for disease pathogenesis

Young SG, Fong LG, Michaelis S. J Lipid Res. 2005;46(12):2531-2558. doi:10.1194/jlr.R500011-JLR200

Novel progerin-interactive partner proteins hnRNP E1, EGF, Mel 18, and UBC9 interact with lamin A/C

Zhong N, Radu G, Ju W, Brown WT. Biochem Biophys Res Commun. 2005;338(2):855-861. doi:10.1016/j.bbrc.2005.10.020

2004

Genome-scale expression profiling of Hutchinson-Gilford progeria syndrome reveals widespread transcriptional misregulation leading to mesodermal/mesenchymal defects and accelerated atherosclerosis

Csoka AB, English SB, Simkevich CP, et al. Aging Cell. 2004;3(4):235-243. doi:10.1111/j.1474-9728.2004.00105.x

Heterozygosity for Lmna deficiency eliminates the progeria-like phenotypes in Zmpste24-deficient mice

Fong LG, Ng JK, Meta M, et al. Proc Natl Acad Sci U S A. 2004;101(52):18111-18116. doi:10.1073/pnas.0408558102

Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndrome

Goldman RD, Shumaker DK, Erdos MR, et al. Proc Natl Acad Sci U S A. 2004;101(24):8963-8968. doi:10.1073/pnas.0402943101

2003

LMNA mutations in atypical Werner’s syndrome

Chen L, Lee L, Kudlow BA, et al. Lancet. 2003;362(9382):440-445. doi:10.1016/S0140-6736(03)14069-X

Recurrent de novo point mutations in lamin A cause Hutchinson-Gilford progeria syndrome

Eriksson M, Brown WT, Gordon LB, et al. Nature. 2003;423(6937):293-298. doi:10.1038/nature01629

Hyaluronan is not elevated in urine or serum in Hutchinson-Gilford Progeria Syndrome

Gordon LB, Harten IA, Calabro A, et al. Hum Genet. 2003;113(2):178-187. doi:10.1007/s00439-003-0958-9

2002

Searching for clues to premature aging

Uitto J. Trends Endocrinol Metab. 2002;13(4):140-141. doi:10.1016/s1043-2760(02)00595-7