Is the oxidant/antioxidant status altered in CADASIL patients?

The altered aggregation of proteins in non-native conformation is associated with endoplasmic reticulum derangements, mitochondrial dysfunction and excessive production of reactive oxygen species. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is...

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Autores principales:	Jonica Campolo, Renata De Maria, Caterina Mariotti, Chiara Tomasello, Marina Parolini, Marina Frontali, Domenico Inzitari, Raffaella Valenti, Antonio Federico, Franco Taroni, Oberdan Parodi
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2013
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/d59174d38d7c480880ba63caa8e5b2c8
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spelling	oai:doaj.org-article:d59174d38d7c480880ba63caa8e5b2c82021-11-18T07:41:36ZIs the oxidant/antioxidant status altered in CADASIL patients?1932-620310.1371/journal.pone.0067077https://doaj.org/article/d59174d38d7c480880ba63caa8e5b2c82013-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/23799141/?tool=EBIhttps://doaj.org/toc/1932-6203The altered aggregation of proteins in non-native conformation is associated with endoplasmic reticulum derangements, mitochondrial dysfunction and excessive production of reactive oxygen species. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare hereditary systemic vasculopathy, caused by NOTCH3 mutations within the receptor extracellular domain, that lead to abnormal accumulation of the mutated protein in the vascular wall. NOTCH3 misfolding could cause free radicals increase also in CADASIL. Aim of the study was to verify whether CADASIL patients have increased oxidative stress compared to unrelated healthy controls. We enrolled 15 CADASIL patients and 16 gender- and age-matched healthy controls with comparable cardiovascular risk factor. Blood and plasma reduced and total aminothiols (homocysteine, cysteine, glutathione, cysteinylglycine) were measured by HPLC and plasma 3-nitrotyrosine by ELISA. Only plasma reduced cysteine (Pr-Cys) and blood reduced glutathione (Br-GSH) concentrations differed between groups: in CADASIL patients Br-GSH levels were higher (p = 0.019) and Pr-Cys lower (p = 0.010) than in controls. No correlation was found between Br-GSH and Pr-Cys either in CADASIL patients (rho 0.25, P = 0.36) or in controls (rho -0.15, P = 0.44). Conversely, 3-nitrotyrosine values were similar in CADASIL and healthy subjects (p = 0.82). The high levels of antioxidant molecules and low levels of oxidant mediators found in our CADASIL population might either be expression of an effective protective action against free radical formation at an early stage of clinical symptoms or they could suggest that oxidative stress is not directly involved in the pathogenesis of CADASIL.Jonica CampoloRenata De MariaCaterina MariottiChiara TomaselloMarina ParoliniMarina FrontaliDomenico InzitariRaffaella ValentiAntonio FedericoFranco TaroniOberdan ParodiPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 8, Iss 6, p e67077 (2013)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Jonica Campolo Renata De Maria Caterina Mariotti Chiara Tomasello Marina Parolini Marina Frontali Domenico Inzitari Raffaella Valenti Antonio Federico Franco Taroni Oberdan Parodi Is the oxidant/antioxidant status altered in CADASIL patients?
description	The altered aggregation of proteins in non-native conformation is associated with endoplasmic reticulum derangements, mitochondrial dysfunction and excessive production of reactive oxygen species. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare hereditary systemic vasculopathy, caused by NOTCH3 mutations within the receptor extracellular domain, that lead to abnormal accumulation of the mutated protein in the vascular wall. NOTCH3 misfolding could cause free radicals increase also in CADASIL. Aim of the study was to verify whether CADASIL patients have increased oxidative stress compared to unrelated healthy controls. We enrolled 15 CADASIL patients and 16 gender- and age-matched healthy controls with comparable cardiovascular risk factor. Blood and plasma reduced and total aminothiols (homocysteine, cysteine, glutathione, cysteinylglycine) were measured by HPLC and plasma 3-nitrotyrosine by ELISA. Only plasma reduced cysteine (Pr-Cys) and blood reduced glutathione (Br-GSH) concentrations differed between groups: in CADASIL patients Br-GSH levels were higher (p = 0.019) and Pr-Cys lower (p = 0.010) than in controls. No correlation was found between Br-GSH and Pr-Cys either in CADASIL patients (rho 0.25, P = 0.36) or in controls (rho -0.15, P = 0.44). Conversely, 3-nitrotyrosine values were similar in CADASIL and healthy subjects (p = 0.82). The high levels of antioxidant molecules and low levels of oxidant mediators found in our CADASIL population might either be expression of an effective protective action against free radical formation at an early stage of clinical symptoms or they could suggest that oxidative stress is not directly involved in the pathogenesis of CADASIL.
format	article
author	Jonica Campolo Renata De Maria Caterina Mariotti Chiara Tomasello Marina Parolini Marina Frontali Domenico Inzitari Raffaella Valenti Antonio Federico Franco Taroni Oberdan Parodi
author_facet	Jonica Campolo Renata De Maria Caterina Mariotti Chiara Tomasello Marina Parolini Marina Frontali Domenico Inzitari Raffaella Valenti Antonio Federico Franco Taroni Oberdan Parodi
author_sort	Jonica Campolo
title	Is the oxidant/antioxidant status altered in CADASIL patients?
title_short	Is the oxidant/antioxidant status altered in CADASIL patients?
title_full	Is the oxidant/antioxidant status altered in CADASIL patients?
title_fullStr	Is the oxidant/antioxidant status altered in CADASIL patients?
title_full_unstemmed	Is the oxidant/antioxidant status altered in CADASIL patients?
title_sort	is the oxidant/antioxidant status altered in cadasil patients?
publisher	Public Library of Science (PLoS)
publishDate	2013
url	https://doaj.org/article/d59174d38d7c480880ba63caa8e5b2c8
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Is the oxidant/antioxidant status altered in CADASIL patients?

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