Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Arterial remodelling in Fabry disease.

P Boutouyrie1, S Laurent, B Laloux

  • 1Department of Pharmacology, INSERM EMI 107, Hôpital Européen Georges Pompidou, Paris, France.

Acta Paediatrica (Oslo, Norway : 1992). Supplement
|February 8, 2003
PubMed
Summary

Fabry disease significantly increases arterial wall thickness and distensibility in medium-sized arteries. This finding highlights potential impacts on large artery health in patients with this genetic disorder.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Clusters of vascular aging manifestations predict incident cardiovascular events in the community.

Nature communications·2026
Same author

Change in Arterial Stiffness Is Associated With Change in Blood Glucose: Longitudinal Study in the Whitehall II Cohort.

Arteriosclerosis, thrombosis, and vascular biology·2025
Same author

Accelerated Vascular Aging as a Possible Mechanism of Troponin I Release in the Absence of Clinically Manifested Myocardial Injury.

Journal of the American Heart Association·2025
Same author

[Thoracic calcifications].

La Revue de medecine interne·2023
Same author

Obstructive sleep apnea, circulating microRNAs, and risk of cardiovascular disease.

Sleep medicine·2023
Same author

[Pulmonary phenotypes of inborn errors of metabolism].

Revue des maladies respiratoires·2022

Area of Science:

  • Vascular biology
  • Cardiovascular medicine
  • Rare genetic diseases

Background:

  • Fabry disease, caused by an enzymatic defect, leads to glycosphingolipid accumulation in vascular cells.
  • While small vessel effects are known, large artery impact in Fabry disease remains under-investigated.

Purpose of the Study:

  • To compare arterial wall structure and function in Fabry disease patients versus age-matched controls.
  • To investigate the effects of Fabry disease on peripheral large arteries.

Main Methods:

  • Non-invasive assessment of large artery phenotype in 21 hemizygous Fabry patients and 24 controls.
  • Utilized high-definition echotracking and tonometry to measure carotid and radial artery diameter, intima-media thickness (IMT), and distensibility.

Related Experiment Videos

Main Results:

  • Fabry patients showed a twofold increase in radial artery IMT and distensibility, independent of other factors.
  • Radial artery IMT increased more significantly with age in Fabry patients compared to controls.
  • Carotid artery IMT was mildly increased in Fabry patients, while distensibility remained unchanged.

Conclusions:

  • This study demonstrates a significant increase in arterial wall thickness and distensibility in medium-sized arteries of Fabry disease patients.
  • Findings suggest Fabry disease has a notable impact on peripheral large artery structure and function.