Progress and trends in the development of therapies for Hutchinson–Gilford progeria syndrome

Lamin A buffers CK2 kinase activity to modulate aging in a progeria mouse model.

(2019) Ying Ao et al.   Science Advances

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

Tackling Aging by Using miRNA as a Target and a Tool

(2019) Wing-Fu Lai et al.   TRENDS IN MOLECULAR MEDICINE

Are There Common Mechanisms Between the Hutchinson–Gilford Progeria Syndrome and Natural Aging?

(2019) Vasily V. Ashapkin et al.   Frontiers in Genetics

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

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

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

(2019) Ya-Hsuan Ho et al.   Cell Stem Cell

Healthspan and lifespan extension by fecal microbiota transplantation into progeroid mice

(2019) Clea Bárcena et al.   NATURE MEDICINE

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

(2019) Ricardo Villa-Bellosta   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Inhibition of DNA damage response at telomeres improves the detrimental phenotypes of Hutchinson–Gilford Progeria Syndrome

(2019) Julio Aguado et al.   Nature Communications

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

(2019) Liu et al.   Cells

Farnesyltransferase inhibitor and rapamycin correct aberrant genome organisation and decrease DNA damage respectively, in Hutchinson–Gilford progeria syndrome fibroblasts

(2018) Mehmet U. Bikkul et al.   BIOGERONTOLOGY

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

(2018) Leslie B. Gordon et al.   JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION

Targeting the phospholipase A2 receptor ameliorates premature aging phenotypes

(2018) Audrey Griveau et al.   AGING CELL

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

(2018) Clea Bárcena et al.   Cell Reports

Chemical screen identifies a geroprotective role of quercetin in premature aging

(2018) Lingling Geng et al.   Protein & Cell

Next-Generation Sequencing and Quantitative Proteomics of Hutchinson-Gilford progeria syndrome-derived cells point to a role of nucleotide metabolism in premature aging

(2018) Jesús Mateos et al.   PLoS One

Impaired immune surveillance accelerates accumulation of senescent cells and aging

(2018) Yossi Ovadya et al.   Nature Communications

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

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

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

(2017) Diana Gabriel et al.   Oncotarget

Temsirolimus Partially Rescues the Hutchinson-Gilford Progeria Cellular Phenotype

(2017) Diana Gabriel et al.   PLoS One

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

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

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

(2016) Su-Jin Lee et al.   JOURNAL OF CLINICAL INVESTIGATION

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

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

Rapamycin: An InhibiTOR of Aging Emerges From the Soil of Easter Island

(2016) Sebastian I. Arriola Apelo et al.   JOURNALS OF GERONTOLOGY SERIES A-BIOLOGICAL SCIENCES AND MEDICAL SCIENCES

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

(2016) Ray Kreienkamp et al.   Oncotarget

Antisense-Based Progerin Downregulation in HGPS-Like Patients’ Cells

(2016) Karim Harhouri et al.   Cells

Methylene blue alleviates nuclear and mitochondrial abnormalities in progeria

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

Hutchinson–Gilford progeria syndrome as a model for vascular aging

(2015) Jonathan A. Brassard et al.   BIOGERONTOLOGY

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

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

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

(2015) Charlotte Strandgren et al.   FASEB JOURNAL

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

Progerin reduces LAP2α-telomere association in Hutchinson-Gilford progeria

(2015) Alexandre Chojnowski et al.   eLife

Sulforaphane enhances progerin clearance in Hutchinson-Gilford progeria fibroblasts

(2014) Diana Gabriel et al.   AGING CELL

Blocking protein farnesylation improves nuclear shape abnormalities in keratinocytes of mice expressing the prelamin A variant in Hutchinson-Gilford progeria syndrome

(2014) Yuexia Wang et al.   Nucleus

Hutchinson-Gilford progeria syndrome through the lens of transcription

(2013) Miron Prokocimer et al.   AGING 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

N6-isopentenyladenosine improves nuclear shape in fibroblasts from humans with progeroid syndromes by inhibiting the farnesylation of prelamin A

(2013) Maurizio Bifulco et al.   FEBS Journal

Neurologic features of Hutchinson-Gilford progeria syndrome after lonafarnib treatment

(2013) N. J. Ullrich et al.   NEUROLOGY

Targeting Isoprenylcysteine Methylation Ameliorates Disease in a Mouse Model of Progeria

(2013) M. X. Ibrahim et al.   SCIENCE

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

(2013) Baohua Liu et al.   Nature Communications

Nucleic acid delivery: Roles in biogerontological interventions

(2012) Wing-Fu Lai   AGEING RESEARCH REVIEWS

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

(2012) Baohua Liu et al.   Cell Metabolism

Protein kinases as targets for interventive biogerontology: Overview and perspectives

(2012) Wing-Fu Lai   EXPERIMENTAL GERONTOLOGY

Nuclear lamina defects cause ATM-dependent NF- B activation and link accelerated aging to a systemic inflammatory response

(2012) F. G. Osorio et al.   GENES & DEVELOPMENT

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

Targeted Gene Correction of Laminopathy-Associated LMNA Mutations in Patient-Specific iPSCs

(2011) Guang-Hui Liu et al.   Cell Stem Cell

Autophagic degradation of farnesylated prelamin A as a therapeutic approach to lamin-linked progeria

(2011) V. Cenni et al.   EUROPEAN JOURNAL OF HISTOCHEMISTRY

The accumulation of un-repairable DNA damage in laminopathy progeria fibroblasts is caused by ROS generation and is prevented by treatment with N-acetyl cysteine

(2011) Shane A. Richards et al.   HUMAN MOLECULAR GENETICS

Nucleic acid therapy for lifespan prolongation: Present and future

(2011) Wing-Fu Lai   JOURNAL OF BIOSCIENCES

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

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

Farnesyltransferase inhibitor treatment restores chromosome territory positions and active chromosome dynamics in Hutchinson-Gilford progeria syndrome cells

(2011) Ishita S Mehta et al.   GENOME BIOLOGY

A Human iPSC Model of Hutchinson Gilford Progeria Reveals Vascular Smooth Muscle and Mesenchymal Stem Cell Defects

(2010) Jinqiu Zhang et al.   Cell Stem Cell

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

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

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

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

Stem-Cell Gene Therapy for the Wiskott–Aldrich Syndrome

(2010) Kaan Boztug et al.   NEW ENGLAND JOURNAL OF MEDICINE

Insulin-like growth factor 1 treatment extends longevity in a mouse model of human premature aging by restoring somatotroph axis function

(2010) G. Marino et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Insertional oncogenesis in 4 patients after retrovirus-mediated gene therapy of SCID-X1

(2008) Salima Hacein-Bey-Abina et al.   JOURNAL OF CLINICAL INVESTIGATION

Suppression of Proliferative Defects Associated with Processing-defective Lamin A Mutants by hTERT or Inactivation of p53

(2008) Brian A. Kudlow et al.   MOLECULAR BIOLOGY OF THE CELL

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