Nitric Oxide Synthesis Is Increased in Cybrid Cells with m.3243A > G Mutation

Página do item simplificado
dc.contributor.authorGamba, Juliana [UNIFESP]
dc.contributor.authorGamba, Luana Tesser [UNIFESP]
dc.contributor.authorRodrigues, Gabriela S. [UNIFESP]
dc.contributor.authorKiyomoto, Beatriz Hitomi [UNIFESP]
dc.contributor.authorMoraes, Carlos T.
dc.contributor.authorTengan, Celia Harumi [UNIFESP]
dc.contributor.institutionUniversidade Federal de São Paulo (UNIFESP)
dc.contributor.institutionUniv Miami
dc.date.accessioned2016-01-24T14:30:57Z
dc.date.available2016-01-24T14:30:57Z
dc.date.issued2013-01-01
dc.description.abstractNitric oxide (NO) is a free radical and a signaling molecule in several pathways, produced by nitric oxide synthase (NOS) from the conversion of L-arginine to citrulline. Supplementation of L-arginine has been used to treat MELAS (mitochondrial encephalopathy with lactic acidosis and stroke like syndrome), a mitochondrial disease caused by the m. 3243A>G mutation. Low levels of serum arginine and endothelium dysfunction have been reported in MELAS and this treatment may increase NO in endothelial cells and promote vasodilation, decreasing cerebral ischemia and strokes. Although clinical benefits have been reported, little is known about NO synthesis in MELAS. in this study we found that osteosarcoma derived cybrid cells with high levels of m. 3243A>G had increased nitrite, an NO metabolite, and increased intracellular NO, demonstrated by an NO fluorescent probe (DAF-FM). Muscle vessels from patients with the same mutation had increased staining in NADPH diaphorase, suggestive of increased NOS. These results indicate increased production of NO in cells harboring the m. 3243A>G, however no nitrated protein was detected by Western blotting. Further studies are necessary to clarify the exact mechanisms of L-arginine effect to determine the appropriate clinical use of this drug therapy.en
dc.description.affiliationUniversidade Federal de São Paulo, Escola Paulista Med, Dept Neurol & Neurosurg, BR-04039032 São Paulo, Brazil
dc.description.affiliationUniv Miami, Miller Sch Med, Dept Neurol & Cell Biol, Miami, FL 33101 USA
dc.description.affiliationUnifespUniversidade Federal de São Paulo, Escola Paulista Med, Dept Neurol & Neurosurg, BR-04039032 São Paulo, Brazil
dc.description.sourceWeb of Science
dc.description.sponsorshipFundacao de Amparo a Pesquisa de São Paulo
dc.description.sponsorshipCoordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)
dc.format.extent394-410
dc.identifierhttp://dx.doi.org/10.3390/ijms14010394
dc.identifier.citationInternational Journal of Molecular Sciences. Basel: Mdpi Ag, v. 14, n. 1, p. 394-410, 2013.
dc.identifier.doi10.3390/ijms14010394
dc.identifier.fileWOS000314048800024.pdf
dc.identifier.issn1422-0067
dc.identifier.urihttp://repositorio.unifesp.br/handle/11600/35740
dc.identifier.wosWOS:000314048800024
dc.language.isoeng
dc.publisherMdpi Ag
dc.relation.ispartofInternational Journal of Molecular Sciences
dc.rightsAcesso aberto
dc.subjectmitochondriaen
dc.subjectnitric oxideen
dc.subjectarginineen
dc.subjectmitochondrial diseaseen
dc.subjectmitochondrial DNAen
dc.titleNitric Oxide Synthesis Is Increased in Cybrid Cells with m.3243A > G Mutationen
dc.typeArtigo
Arquivos
Pacote Original
Agora exibindo 1 - 1 de 1
Imagem de Miniatura
Nome:
WOS000314048800024.pdf
Tamanho:
1.02 MB
Formato:
Adobe Portable Document Format
Descrição:
Baixar
Coleções
EPM - Artigos