Glutathione system participation in thoracic aneurysms from patients with Marfan syndrome.
BACKGROUND
Aortic dilatation in Marfan syndrome (MFS) is progressive. It is associated with oxidative stress and endothelial dysfunction that contribute to the early acute dissection of the vessel and can result in rupture of the aorta and sudden death. We evaluated the participation of the glutathione (GSH) system, which could be involved in the mechanisms that promote the formation and progression of the aortic aneurysms in MFS patients.


METHODS
Aortic aneurysm tissue was obtained during chest surgery from eight control subjects and 14 MFS patients. Spectrophotometrical determination of activity of glutathione peroxidase (GPx), glutathione-S-transferase (GST), glutathione reductase (GR), lipid peroxidation (LPO) index, carbonylation, total antioxidant capacity (TAC), and concentration of reduced and oxidized glutathione (GSH and GSSG respectively), was performed in the homogenate from aortic aneurysm tissue.


RESULTS
LPO index, carbonylation, TGF-β1, and GR activity were increased in MFS patients (p < 0.04), while TAC, GSH/GSSG ratio, GPx, and GST activity were significantly decreased (p < 0.04).


CONCLUSIONS
The depletion of GSH, in spite of the elevated activity of GR, not only diminished the activity of GSH-depend GST and GPx, but increased LPO, carbonylation and decreased TAC. These changes could promote the structural and functional alterations in the thoracic aorta of MFS patients.
2 Pathology, Instituto Nacional de Cardiología Ignacio Chávez, México City, México.
Alejandra María
AM
Zúñiga-Muñoz
2 Pathology, Instituto Nacional de Cardiología Ignacio Chávez, México City, México.
Israel
I
Pérez-Torres
3 Physiology, Instituto Nacional de Cardiología Ignacio Chávez, México City, México.
Verónica
V
Guarner-Lans
4 Cardiothoracic Surgery, Instituto Nacional de Cardiología Ignacio Chávez, México City, México.
Elías
E
Núñez-Garrido
2 Pathology, Instituto Nacional de Cardiología Ignacio Chávez, México City, México.
Rodrigo
R
Velázquez Espejel
3 Physiology, Instituto Nacional de Cardiología Ignacio Chávez, México City, México.
Claudia
C
Huesca-Gómez
3 Physiology, Instituto Nacional de Cardiología Ignacio Chávez, México City, México.
Ricardo
R
Gamboa-Ávila
1 Departaments of Immunology, Instituto Nacional de Cardiología Ignacio Chávez, México City, México.
María Elena
ME
Soto
D000328:Adult; D000368:Aged; D001013:Aorta, Thoracic / Q000737:chemistry* / Q000473:pathology; D017545:Aortic Aneurysm, Thoracic / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism; D015415:Biomarkers / Q000032:analysis; D016022:Case-Control Studies; D002648:Child; D004108:Dilatation, Pathologic; D005260:Female; D005978:Glutathione / Q000032:analysis*; D005979:Glutathione Peroxidase / Q000032:analysis; D005980:Glutathione Reductase / Q000032:analysis; D005982:Glutathione Transferase / Q000032:analysis; D006801:Humans; D015227:Lipid Peroxidation; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism; D008875:Middle Aged; D018384:Oxidative Stress; D011446:Prospective Studies; D050050:Protein Carbonylation; D053773:Transforming Growth Factor beta1 / Q000032:analysis
D015415:Biomarkers; C508960:TGFB1 protein, human; D053773:Transforming Growth Factor beta1; D005979:Glutathione Peroxidase; D005980:Glutathione Reductase; D005982:Glutathione Transferase; D005978:Glutathione
Glutathione; Marfan syndrome; aneurysm; oxidative stress; thoracic aorta; vascular disease