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dc.contributor.authorVanzin, Camila Simionipt_BR
dc.contributor.authorMescka, Caroline Paulapt_BR
dc.contributor.authorMarchetti, Desirèe Padilhapt_BR
dc.contributor.authorDonida, Brunapt_BR
dc.contributor.authorDeon, Marionpt_BR
dc.contributor.authorJacques, Carlos Eduardo Diazpt_BR
dc.contributor.authorHauschild, Tatiane Cristinapt_BR
dc.contributor.authorFaverzani, Jéssica Lambertypt_BR
dc.contributor.authorHauschild, Tatiane Cristinapt_BR
dc.contributor.authorMoura, Dinara Jaquelinept_BR
dc.contributor.authorSaffi, Jeniferpt_BR
dc.contributor.authorCoelho, Daniella de Mourapt_BR
dc.contributor.authorWajner, Moacirpt_BR
dc.contributor.authorWyse, Angela Terezinha de Souzapt_BR
dc.contributor.authorVargas, Carmen Reglapt_BR
dc.date.accessioned2018-04-20T02:30:40Zpt_BR
dc.date.issued2018pt_BR
dc.identifier.issn2357-9730pt_BR
dc.identifier.urihttp://hdl.handle.net/10183/174878pt_BR
dc.description.abstractIntroduction: Homocysteine (Hcy) tissue accumulation occurs in a metabolic disease characterized biochemically by cystathionine β-synthase (CBS) deficiency and clinically by mental retardation, vascular problems, and skeletal abnormalities. Previous studies indicate the occurrence of DNA damage secondary to hyperhomocysteinemia and it was observed that DNA damage occurs in leukocytes from CBS-deficient patients. This study aimed to investigate whether an oxidative mechanism could be involved in DNA damage previously found and investigated the in vitro effect of N-acety-L-cysteine (NAC) on DNA damage caused by high Hcy levels. Methods: We evaluated a biomarker of oxidative DNA damage in the urine of CBS‑deficient patients, as well as the in vitro effect of NAC on DNA damage caused by high levels of Hcy. Moreover, a biomarker of lipid oxidative damage was also measured in urine of CBS deficient patients. Results: There was an increase in parameters of DNA (8-oxo-7,8-dihydro-2’- deoxyguanosine) and lipid (15-F2t-isoprostanes levels) oxidative damage in CBS-deficient patients when compared to controls. In addition, a significant positive correlation was found between 15-F2t-isoprostanes levels and total Hcy concentrations. Besides, an in vitro protective effect of NAC at concentrations of 1 and 5 mM was observed on DNA damage caused by Hcy 50 μM and 200 μM. Additionally, we showed a decrease in sulfhydryl content in plasma from CBS-deficient patients when compared to controls. Discussion: These results demonstrated that DNA damage occurs by an oxidative mechanism in CBS deficiency together with lipid oxidative damage, highlighting the NAC beneficial action upon DNA oxidative process, contributing with a new treatment perspective of the patients affected by classic homocystinuria.en
dc.format.mimetypeapplication/pdfpt_BR
dc.language.isoengpt_BR
dc.relation.ispartofClinical and biomedical research. Porto Alegre, RS. Vol. 38, no. 1 (2018), p. 50-57pt_BR
dc.rightsOpen Accessen
dc.subjectN-Acetil-L-Cisteínapt_BR
dc.subjectCystathionine-β-synthase deficiencyen
dc.subjectOxidative stressen
dc.subjectHomocistinúriapt_BR
dc.subject8-oxo-7en
dc.subject7,8-dihydro- 2’-deoxyguanosineen
dc.subjectHomocysteineen
dc.subjectDNA damageen
dc.subjectN-acetyl-L-cysteineen
dc.titleDNA damage in homocystinuria : 8-oxo-, 8-dihydro-2’-deoxyguanosine levels in cystathionine-β-synthase deficient patients and the in vitro protective effect of N-acetyl-L-cysteinept_BR
dc.typeArtigo de periódicopt_BR
dc.identifier.nrb001065410pt_BR
dc.type.originNacionalpt_BR


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