Deficiency of the mitochondrial sulfide regulator ETHE1 disturbs cell growth, glutathione level and causes proteome alterations outside mitochondria.

Sahebekhtiari, Navid; Fernandez-Guerra, Paula; Nochi, Zahra; Carlsen, Jasper; Bross, Peter; Palmfeldt, Johan

Sahebekhtiari, Navid; Fernandez-Guerra, Paula; Nochi, Zahra; Carlsen, Jasper; Bross, Peter; Palmfeldt, Johan.

Afiliación

Sahebekhtiari N; Research Unit for Molecular Medicine, Department of Clinical Medicine, Aarhus University and Aarhus University Hospital, Palle Juul-Jensens Boulevard 99, DK-8200 Aarhus N, Denmark.
Fernandez-Guerra P; Research Unit for Molecular Medicine, Department of Clinical Medicine, Aarhus University and Aarhus University Hospital, Palle Juul-Jensens Boulevard 99, DK-8200 Aarhus N, Denmark.
Nochi Z; Research Unit for Molecular Medicine, Department of Clinical Medicine, Aarhus University and Aarhus University Hospital, Palle Juul-Jensens Boulevard 99, DK-8200 Aarhus N, Denmark.
Carlsen J; Research Unit for Molecular Medicine, Department of Clinical Medicine, Aarhus University and Aarhus University Hospital, Palle Juul-Jensens Boulevard 99, DK-8200 Aarhus N, Denmark.
Bross P; Research Unit for Molecular Medicine, Department of Clinical Medicine, Aarhus University and Aarhus University Hospital, Palle Juul-Jensens Boulevard 99, DK-8200 Aarhus N, Denmark.
Palmfeldt J; Research Unit for Molecular Medicine, Department of Clinical Medicine, Aarhus University and Aarhus University Hospital, Palle Juul-Jensens Boulevard 99, DK-8200 Aarhus N, Denmark. Electronic address: johan.palmfeldt@clin.au.dk.

Biochim Biophys Acta Mol Basis Dis ; 1865(1): 126-135, 2019 01.

Article en En | MEDLINE | ID: mdl-30391543

Asunto(s)

Ciclo Celular/fisiología; Mitocondrias/metabolismo; Proteínas Mitocondriales/metabolismo; Proteínas de Transporte Nucleocitoplasmático/metabolismo; Proteoma/metabolismo; Sulfuros/metabolismo; Encefalopatías Metabólicas Innatas; Adhesión Celular; Regulación hacia Abajo; Retículo Endoplásmico/metabolismo; Fibroblastos/metabolismo; Glutatión/metabolismo; Glucólisis; Glicoproteínas/metabolismo; Humanos; Proteínas con Dominio LIM/metabolismo; Ácido Láctico/metabolismo; Masculino; Peroxirredoxinas/metabolismo; Proteína Disulfuro Isomerasas/metabolismo; Proteómica; Púrpura; Proteínas Ribosómicas

Palabras clave

Ethylmalonic encephalopathy; Glutathione; Glycosylation; Mitochondrion; Redox regulation; Sulfide

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Texto completo: 1 Base de datos: MEDLINE Asunto principal: Sulfuros / Ciclo Celular / Proteoma / Proteínas de Transporte Nucleocitoplasmático / Proteínas Mitocondriales / Mitocondrias Tipo de estudio: Etiology_studies Límite: Humans / Male Idioma: En Revista: Biochim Biophys Acta Mol Basis Dis Año: 2019 Tipo del documento: Article País de afiliación: Dinamarca

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