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Oxidant/Antioxidant Profile in the Thoracic Aneurysm of Patients with the Loeys-Dietz Syndrome.

ABSTRACT: Patients with the Loeys-Dietz syndrome (LDS) have mutations in the TGF-?R1, TGF-?R2, and SMAD3 genes. However, little is known about the redox homeostasis in the thoracic aortic aneurysms (TAA) they develop. Here, we evaluate the oxidant/antioxidant profile in the TAA tissue from LDS patients and compare it with that in nondamaged aortic tissue from control (C) subjects. We evaluate the enzymatic activities of glutathione peroxidase (GPx), glutathione S-transferase (GST), glutathione reductase (GR), catalase (CAT), superoxide dismutase (SOD) isoforms, and thioredoxin reductase (TrxR). We also analyze some antioxidants from a nonenzymatic system such as selenium (Se), glutathione (GSH), and total antioxidant capacity (TAC). Oxidative stress markers such as lipid peroxidation and carbonylation, as well as xanthine oxidase (ORX) and nuclear factor erythroid 2-related factor 2 (Nrf2) expressions, were also evaluated. TAA from LDS patients showed a decrease in GSH, Se, TAC, GPx, GST, CAT, and TrxR. The SOD activity and ORX expressions were increased, but the Nrf2 expression was decreased. The results suggest that the redox homeostasis is altered in the TAA from LDS patients, favoring ROS overproduction that contributes to the decrease in GSH and TAC and leads to LPO and carbonylation. The decrease in Se and Nrf2 alters the activity and/or expression of some antioxidant enzymes, thus favoring a positive feedback oxidative background that contributes to the TAA formation.

SUBMITTER: Soto ME

PROVIDER: S-EPMC7128053 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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Oxidant/Antioxidant Profile in the Thoracic Aneurysm of Patients with the Loeys-Dietz Syndrome.

Soto Maria Elena ME Manzano-Pech Lináloe G LG Guarner-Lans Verónica V Díaz-Galindo Jorge A JA Vásquez Xicoténcatl X Castrejón-Tellez Vicente V Gamboa Ricardo R Huesca Claudia C Fuentevilla-Alvárez Giovanny G Pérez-Torres Israel I

Oxidative medicine and cellular longevity 20200323

Patients with the Loeys-Dietz syndrome (LDS) have mutations in the TGF-<i>β</i>R1, TGF-<i>β</i>R2, and SMAD3 genes. However, little is known about the redox homeostasis in the thoracic aortic aneurysms (TAA) they develop. Here, we evaluate the oxidant/antioxidant profile in the TAA tissue from LDS patients and compare it with that in nondamaged aortic tissue from control (C) subjects. We evaluate the enzymatic activities of glutathione peroxidase (GPx), glutathione S-transferase (GST), glutathio ...[more]

PMID: 32273946

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Project description:Background and purposeLoeys-Dietz syndrome (LDS) is a recently described entity that has the triad of arterial tortuosity and aneurysms, hypertelorism, and bifid uvula or cleft palate. Its neuroradiologic manifestations have not been well delineated. We sought to describe the neuroradiologic features of LDS and to assess the manifestations that would warrant follow-up imaging.Materials and methodsTwo neuroradiologists retrospectively reviewed CT angiography (CTA), MR imaging, and plain film studies related to the head and neck in 25 patients ranging from 1 to 55 years of age, all of whom had positive genetic testing and clinical characteristics of LDS. Arterial tortuosity was evaluated by subjective assessment of 2D and 3D volumetric CTA and MR angiography data. Craniosynostosis and spinal manifestations were assessed by using plain films and CT images. MR images mostly of the head were reviewed for associated findings such as hydrocephalus, Chiari malformation, etc. Clinical manifestations were collated from the electronic patient record.ResultsAll patients had extreme arterial tortuosity, which is characteristic of this syndrome. Thirteen patients had scoliosis, 12 had craniosynostosis, 8 had intracranial aneurysms, 6 had spinal instability, 3 had dissections of the carotid and vertebrobasilar arteries, 3 had hydrocephalus, 4 had dural ectasia, 2 had a Chiari malformation, and 1 had intracranial hemorrhage as a complication of vascular dissection.ConclusionsSignificant neuroradiologic manifestations are associated with LDS, predominantly arterial tortuosity. Most of the patients in this series were young and, therefore, may require serial CTA monitoring for development of intra- and extracranial dissections and aneurysms, on the basis of the fact that most of the patients with pseudoaneurysms and dissection were older at the time of imaging. Other findings of LDS such as craniosynostosis, Chiari malformation, and spinal instability may also need to be addressed.

Dataset Information

Oxidant/Antioxidant Profile in the Thoracic Aneurysm of Patients with the Loeys-Dietz Syndrome.

Publications

Oxidant/Antioxidant Profile in the Thoracic Aneurysm of Patients with the Loeys-Dietz Syndrome.

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