Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Paradoxical aortic stiffening and subsequent cardiac dysfunction in Hutchinson-Gilford progeria syndrome.

Journal of the Royal Society, Interface - 1 May 2020

Murtada S-I, Kawamura Y, Caulk A W, Ahmadzadeh H, Mikush N, Zimmerman K, Kavanagh D, Weiss D, Latorre M, Zhuang Z W, Shadel G S, Braddock D T, Humphrey J D

Abstract excerpt

Hutchinson-Gilford progeria syndrome (HGPS) is an ultra-rare disorder with devastating sequelae resulting in early death, presently thought to stem primarily from cardiovascular events. We analyse novel longitudinal cardiovascular data from a mouse model of HGPS (LmnaG609G/G609G) using allometric scaling, biomechanical phenotyping, and advanced computational modelling and show that late-stage diastolic...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Aorta
Share to Heart Diseases

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Muscle, Smooth, Vascular

Share to Mutation

Share to Progeria

Share to Pulse Wave Analysis

Source

Open in PubMed Open full text

Identifiers

PMID: 32453981
DOI: 10.1098/rsif.2020.0066
PMCID: PMC7276555

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Paradoxical aortic stiffening and subsequent cardiac dysfunction in Hutchinson-Gilford progeria syndrome.PMID 32453981Read ↗

Paradoxical Aortic Stiffening and Subsequent Cardiac Dysfunction in Hutchinson-Gilford Progeria SyndromeSemantic proximity Read ↗

Lonafarnib improves cardiovascular function and survival in a mouse model of Hutchinson-Gilford progeria syndromeSemantic proximity Read ↗

Lonafarnib improves cardiovascular function and survival in a mouse model of Hutchinson-Gilford Progeria SyndromeSemantic proximity Read ↗

Biomechanical and transcriptional evidence that smooth muscle cell death drives an osteochondrogenic phenotype and severe proximal vascular disease in progeria.Semantic proximity Read ↗

Molecular and Cellular Mechanisms Driving Cardiovascular Disease in Hutchinson-Gilford Progeria Syndrome: Lessons Learned from Animal Models.Semantic proximity Read ↗

Smooth Muscle Cell Death Drives an Osteochondrogenic Phenotype and Severe Proximal Vascular Disease in ProgeriaSemantic proximity Read ↗

Premature Vascular Aging with Features of Plaque Vulnerability in an Atheroprone Mouse Model of Hutchinson-Gilford Progeria Syndrome with Ldlr Deficiency.Semantic proximity Read ↗

Intervention for Critical Aortic Stenosis in Hutchinson-Gilford Progeria SyndromeSemantic proximity Read ↗

Single-cell analysis of the progeria arterial wall reveals progerin-induced progressive, cell type-specific dysfunction and somatic mutation accumulation.Semantic proximity Read ↗

Arterial stiffness and cardiac dysfunction in Hutchinson–Gilford Progeria Syndrome corrected by inhibition of lysyl oxidaseSemantic proximity Read ↗

Nuclear membrane ruptures underlie the vascular pathology in a mouse model of Hutchinson-Gilford progeria syndrome.Semantic proximity Read ↗

Myh11(R247C/R247C) mutations increase thoracic aorta vulnerability to intramural damage despite a general biomechanical adaptivity.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.