Modulation of mitochondrial dysfunction-related oxidative stress in fibroblasts of patients with Leigh syndrome by inhibition of prooxidative p66Shc pathway.

Wojtala, Aleksandra; Karkucinska-Wieckowska, Agnieszka; Sardao, Vilma A; Szczepanowska, Joanna; Kowalski, Pawel; Pronicki, Maciej; Duszynski, Jerzy; Wieckowski, Mariusz R

Wojtala, Aleksandra; Karkucinska-Wieckowska, Agnieszka; Sardao, Vilma A; Szczepanowska, Joanna; Kowalski, Pawel; Pronicki, Maciej; Duszynski, Jerzy; Wieckowski, Mariusz R.

Afiliação

Wojtala A; Department of Biochemistry, Nencki Institute of Experimental Biology, Warsaw, Poland.
Karkucinska-Wieckowska A; Department of Pathology, The Children's Memorial Health Institute, Warsaw, Poland.
Sardao VA; CNC - Center for Neuroscience and Cell Biology, University of Coimbra, UC-Biotech Building, Biocant Park, Cantanhede, Portugal.
Szczepanowska J; Department of Biochemistry, Nencki Institute of Experimental Biology, Warsaw, Poland.
Kowalski P; Department of Medical Genetics, The Children's Memorial Health Institute, Warsaw, Poland.
Pronicki M; Department of Pathology, The Children's Memorial Health Institute, Warsaw, Poland.
Duszynski J; Department of Biochemistry, Nencki Institute of Experimental Biology, Warsaw, Poland.
Wieckowski MR; Department of Biochemistry, Nencki Institute of Experimental Biology, Warsaw, Poland. Electronic address: m.wieckowski@nencki.gov.pl.

Mitochondrion ; 37: 62-79, 2017 11.

Article em En | MEDLINE | ID: mdl-28739512

Assuntos

Fibroblastos/metabolismo; Doença de Leigh/fisiopatologia; Mitocôndrias/metabolismo; Estresse Oxidativo; Espécies Reativas de Oxigênio/metabolismo; Proteína 1 de Transformação que Contém Domínio 2 de Homologia de Src/antagonistas & inibidores; Proteína 1 de Transformação que Contém Domínio 2 de Homologia de Src/metabolismo; Células Cultivadas; Complexo I de Transporte de Elétrons/genética; Inibidores Enzimáticos/metabolismo; Humanos; Mutação; Pironas/metabolismo

Palavras-chave

Complex I deficiency; Hispidin; Leigh syndrome; Mitochondrial dysfunction; Reactive oxygen species

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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Doença de Leigh / Espécies Reativas de Oxigênio / Estresse Oxidativo / Fibroblastos / Proteína 1 de Transformação que Contém Domínio 2 de Homologia de Src / Mitocôndrias Limite: Humans Idioma: En Revista: Mitochondrion Ano de publicação: 2017 Tipo de documento: Article País de afiliação: Polônia

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