ArticleProceedings of the National Academy of Sciences of the United States of America2006
Hutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2006. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 105 papers.
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Who cites it
105 citing papers in PubMed, 195 citations in OpenAlex.
- Clinical trial of a farnesyltransferase inhibitor in children with Hutchinson-Gilford progeria syndrome.Proceedings of the National Academy of Sciences of the United States of America · 2012Trial
- Mechanisms of premature vascular aging in children with Hutchinson-Gilford progeria syndrome.Hypertension (Dallas, Tex. : 1979) · 2012Trial
- Abnormal nuclear morphology in vivo in mouse hair follicle fibroblasts expressing progerin.Biochemical and biophysical research communications · 2026Article
- Nuclear Wrinkles Result From Geometric Adaptations to Cellular and Nuclear Morphological Changes in Epithelial Cells.Genes to cells : devoted to molecular & cellular mechanisms · 2026Article
- Leveraging Artificial Intelligence and Modulation of Oxidative Stressors to Enhance Healthspan and Radical Longevity.Biomolecules · 2025Review
- Myonuclear alterations associated with exercise are independent of age in humans.The Journal of physiology · 2025Article
- Circular RNA Telomerase Reverses Endothelial Senescence in Progeria.Aging cell · 2025Article
- Endothelial cell-specific progerin expression does not cause cardiovascular alterations and premature death.Aging cell · 2025Article
- Tubular Lamin- and Emerin-Lined Nuclear Envelope Invaginations Isolate Chromatin Domains for Gene Expression Regulation.Sub-cellular biochemistry · 2025Review
- Genetic and pharmacological modulation of lamin A farnesylation determines its function and turnover.Aging cell · 2024Article
- Defective prelamin A processing promotes unconventional necroptosis driven by nuclear RIPK1.Nature cell biology · 2024Article
- Progerin, an Aberrant Spliced Form of Lamin A, Is a Potential Therapeutic Target for HGPS.Cells · 2023Review
- Activation of endoplasmic reticulum stress in premature aging via the inner nuclear membrane protein SUN2.Cell reports · 2023Article
- Endothelial senescence in vascular diseases: current understanding and future opportunities in senotherapeutics.Experimental & molecular medicine · 2023Review
- Progeria and Aging-Omics Based Comparative Analysis.Biomedicines · 2022Article
- Vascular Calcification in Chronic Kidney Disease: An Update and Perspective.Aging and disease · 2022Article
- Review
- Review
- The Role of Lamins in the Nucleoplasmic Reticulum, a Pleiomorphic Organelle That Enhances Nucleo-Cytoplasmic Interplay.Frontiers in cell and developmental biology · 2022Review
- Telomerase therapy reverses vascular senescence and extends lifespan in progeria mice.European heart journal · 2021Article
45 more citing papers are in PubMed but not listed here.
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Authors and funding
3 authors at 2 institutions in 1 country.
Funding
Abstract
Hutchinson-Gilford progeria syndrome (HGPS; Online Mendelian Inheritance in Man accession no. 176670) is a rare disorder that is characterized by segmental premature aging and death between 7 and 20 years of age from severe premature atherosclerosis. Mutations in the LMNA gene are responsible for this syndrome. Approximately 80% of HGPS cases are caused by a G608 (GGC-->GGT) mutation within exon 11 of LMNA, which elicits a deletion of 50 aa near the C terminus of prelamin A. In this article, we present evidence that the mutant lamin A (progerin) accumulates in the nucleus in a cellular age-dependent manner. In human HGPS fibroblast cultures, we observed, concomitantly to nuclear progerin accumulation, severe nuclear envelope deformations and invaginations preventable by farnesyltransferase inhibition. Nuclear alterations affect cell-cycle progression and cell migration and elicit premature senescence. Strikingly, skin biopsy sections from a subject with HGPS showed that the truncated lamin A accumulates primarily in the nuclei of vascular cells. This finding suggests that accumulation of progerin is directly involved in vascular disease in progeria.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.