Results 11 to 20 of about 7,451 (172)

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

open access: yesAdvanced Science
Hutchinson‐Gilford progeria syndrome (HGPS) is a rare genetic disorder caused by a mutation in the LMNA gene, leading to the production of progerin, an aberrant and toxic form of lamin A. Due to its hydrophobic nature, progerin accumulates at the nuclear
Jon Macicior‐Michelena   +5 more
doaj   +3 more sources

Progeria

open access: yesIndian Journal of Dental Research, 2009
Hutchinson Gilford Progeria Syndrome (HGPS) is a rare, sporadic, autosomal dominant syndrome that involves premature ageing and death at early age due to myocardial infarction or stroke.
Mohamed Riyaz S, Jayachandran S
doaj   +3 more sources

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

open access: yesStem Cell Reports, 2020
Summary: Hutchinson-Gilford progeria syndrome (HGPS) is a rare disorder caused by a point mutation in the Lamin A gene that produces the protein progerin. Progerin toxicity leads to accelerated aging and death from cardiovascular disease.
Leigh Atchison   +2 more
exaly   +3 more sources

Murine Progeria Model Exhibits Delayed Fracture Healing With Senescent Phenotype and Dysregulated Immune Response [PDF]

open access: yesJournal of Orthopaedic Research, Volume 44, Issue 4, April 2026.
Graphical Abstract This study explores whether a murine model of progeria has a delayed fracture healing phenotype that resembles chronological aging but with an accelerated timeline that could make it a valuable research tool. ABSTRACT An estimated 189 million bone fractures occurred in 2019 making it one of the most globally prevalent injuries ...
Victoria R. Duke   +17 more
wiley   +2 more sources

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

open access: yesEMBO Molecular Medicine, 2021
Inflammation is a hallmark of aging and accelerated aging syndromes such as Hutchinson–Gilford progeria syndrome (HGPS). In this study, we present evidence of increased expression of the components of the NLRP3 inflammasome pathway in HGPS skin ...
Alvaro González-Domínguez   +2 more
exaly   +2 more sources

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

open access: yesMolecular Therapy: Nucleic Acids
Hutchinson-Gilford progeria syndrome is a fatal genetic disorder caused by a point mutation in the gene encoding the nuclear envelope protein lamin A/C.
Volha Dzianisava   +3 more
doaj   +2 more sources

Progeria

open access: yesNursing & Healthcare International Journal
Progeria is a rare genetic disease with striking features that resemble accelerated aging. The inheritance pattern, paternal age effect, and lack of consanguinity argue that it is due to a sporadic dominant mutation. We have observed elevated levels of hyaluronic acid (HA) excretion in progeria patients.
Saleh HM, Sickles CK, Gross GP.
openaire   +2 more sources

Mesenchymal Stem Cell Therapy for Hutchinson–Gilford Progeria: Improvements in Arterial Stiffness and Bone Mineral Density in a Single Case [PDF]

open access: yesChildren
Background/Objectives: Hutchinson–Gilford progeria syndrome (HGPS) is a rare genetic disorder that cause premature aging due to LMNA mutations and progerin accumulation.
Eun-Young Joo   +6 more
doaj   +2 more sources

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

open access: yesNucleus, 2023
Several related progeroid disorders are caused by defective post-translational processing of prelamin A, the precursor of the nuclear scaffold protein lamin A, encoded by LMNA.
Kamsi O. Odinammadu   +5 more
doaj   +1 more source

Progeria Presenting with Pyogenic Granuloma in Conjunctiva: A Case Report

open access: yesJournal of Nepal Medical Association, 2023
Hutchinson-Gilford progeria syndrome frequently exhibits stunted growth and premature ageing. Notable ocular characteristics can encompass a large number of ocular abnormalities.
Sanket Parajuli   +2 more
doaj   +1 more source

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