Related Experiment Video

Updated: Mar 7, 2026

Precision Ultrasound-guided Stem Cell Delivery for Vascular Repair in Aortic Diseases
04:59

Precision Ultrasound-guided Stem Cell Delivery for Vascular Repair in Aortic Diseases

Published on: June 20, 2025

891

Glutathione system participation in thoracic aneurysms from patients with Marfan syndrome

Alejandra María Zúñiga-Muñoz1, Israel Pérez-Torres1, Verónica Guarner-Lans2

  • 12 Pathology, Instituto Nacional de Cardiología Ignacio Chávez, México City, México.

VASA. Zeitschrift Fur Gefasskrankheiten
|February 9, 2017
PubMed
Abstract

Insights

Marfan syndrome (MFS) patients show altered glutathione (GSH) system function, with decreased GSH and related enzyme activity. These changes contribute to aortic aneurysm progression and oxidative stress in MFS.

Area of Science:

  • Biochemistry
  • Cardiovascular Biology
  • Genetics

Background:

  • Marfan syndrome (MFS) is characterized by progressive aortic dilatation, linked to oxidative stress and endothelial dysfunction.
  • These vascular changes increase the risk of aortic dissection, rupture, and sudden death in MFS patients.

Purpose of the Study:

  • To investigate the role of the glutathione (GSH) system in the development and progression of aortic aneurysms in Marfan syndrome.
  • To assess specific enzyme activities and oxidative stress markers within aortic tissue of MFS patients.

Main Methods:

  • Aortic aneurysm tissue samples were collected from 14 MFS patients and 8 control subjects during surgery.
  • Spectrophotometric assays were used to measure the activity of glutathione peroxidase (GPx), glutathione-S-transferase (GST), and glutathione reductase (GR).
  • Levels of lipid peroxidation (LPO) index, carbonylation, total antioxidant capacity (TAC), and reduced/oxidized glutathione (GSH/GSSG) were quantified.

Main Results:

  • MFS patients exhibited elevated LPO index, carbonylation, and GR activity compared to controls (p < 0.04).
  • Significantly decreased TAC, GSH/GSSG ratio, GPx, and GST activity were observed in MFS patients (p < 0.04).

Conclusions:

  • The observed depletion of GSH, despite increased GR activity, impaired the function of GSH-dependent enzymes (GST, GPx).
  • These biochemical alterations, including increased LPO and carbonylation and reduced TAC, likely contribute to the structural and functional deterioration of the aorta in Marfan syndrome.
Keywords:
GlutathioneMarfan syndromeaneurysmoxidative stressthoracic aortavascular disease

More Related Videos

Murine Model of Thoracic Aortic Dissection Induced by Oral &#946;-Aminopropionitrile and Subcutaneous Angiotensin II Infusion05:31

Murine Model of Thoracic Aortic Dissection Induced by Oral β-Aminopropionitrile and Subcutaneous Angiotensin II Infusion

Published on: May 16, 2025

999
A New Murine Model of Endovascular Aortic Aneurysm Repair08:51

A New Murine Model of Endovascular Aortic Aneurysm Repair

Published on: July 7, 2013

14.9K

Related Concept Videos

Aneurysm I: Introduction01:30

Aneurysm I: Introduction

An aortic aneurysm is a localized outpouching or dilation at a weak point in the artery wall. It may involve different parts of the aorta, such as the abdominal aorta, aortic arch, or thoracic aorta.Etiological factorsSeveral disorders are associated with aortic aneurysms.Congenital causes, such as primary connective tissue disorders like Marfan syndrome, impact the integrity and strength of connective tissues, notably affecting the aorta. Marfan syndrome is a genetic disorder that specifically...
564
Aneurysm II: Clinical Manifestations and Diagnostic Studies01:21

Aneurysm II: Clinical Manifestations and Diagnostic Studies

Thoracic, aortic arch and abdominal aneurysms are significant vascular conditions that can present with various clinical manifestations and lead to serious complications. Understanding these manifestations and the appropriate diagnostic studies is essential for effective management and treatment.Thoracic Aortic AneurysmsThoracic aortic aneurysms often remain asymptomatic until they reach a size that impinges on adjacent structures. They typically cause deep, diffuse chest pain that radiates to...
452
Phase II Reactions: Glutathione Conjugation and Mercapturic Acid Formation01:22

Phase II Reactions: Glutathione Conjugation and Mercapturic Acid Formation

Glutathione, a tripeptide made up of glutamate, cysteine, and glycine, is a critical player in the detoxification of drugs and xenobiotics via a process known as glutathione conjugation or mercapturic acid formation. This phase II biotransformation reaction involves the covalent binding of glutathione to a drug or its metabolite, enhancing the compound's water solubility and enabling its excretion.
Several distinctive characteristics distinguish glutathione conjugation from other phase II...
1.1K
Aneurysm III: Interprofessional Care01:26

Aneurysm III: Interprofessional Care

Aneurysm management involves either conservative medical therapy or surgical intervention, depending on the size and symptoms of the aneurysm. Conservative management is generally reserved for smaller, asymptomatic aneurysms, while larger or symptomatic aneurysms often necessitate surgical repair.Conservative Medical TherapyFor small, asymptomatic aneurysms, particularly abdominal aortic aneurysms (AAA) less than 5.5 centimeters in diameter, conservative medical therapy is recommended. This...
422
Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
973
Thoracic Aorta01:15

Thoracic Aorta

The thoracic section of the aorta begins at the T5 vertebra and extends to the T12 level at the diaphragm, initially progressing through the mediastinum to the left of the spinal column. Throughout its course in the thoracic segment, the thoracic aorta emits various offshoots known collectively as visceral and parietal branches. The branches that predominantly supply blood to visceral organs are termed visceral branches and include bronchial, pericardial, esophageal, and mediastinal arteries,...
2.1K