An X-chromosome linked mouse model (Ndufa1<sup>S55A</sup>) for systemic partial Complex I deficiency for studying predisposition to neurodegeneration and other diseases.

Kim, Chul; Potluri, Prasanth; Khalil, Ahmed; Gaut, Daria; McManus, Meagan; Compton, Shannon; Wallace, Douglas C; Yadava, Nagendra

An X-chromosome linked mouse model (Ndufa1^S55A) for systemic partial Complex I deficiency for studying predisposition to neurodegeneration and other diseases.

Kim, Chul; Potluri, Prasanth; Khalil, Ahmed; Gaut, Daria; McManus, Meagan; Compton, Shannon; Wallace, Douglas C; Yadava, Nagendra.

Afiliação

Kim C; Pioneer Valley Life Sciences Institute, Springfield, MA 01199, USA.
Potluri P; Center for Mitochondrial and Epigenomic Medicine at the Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Khalil A; Pioneer Valley Life Sciences Institute, Springfield, MA 01199, USA.
Gaut D; Center for Mitochondrial and Epigenomic Medicine at the Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
McManus M; Center for Mitochondrial and Epigenomic Medicine at the Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Compton S; Pioneer Valley Life Sciences Institute, Springfield, MA 01199, USA.
Wallace DC; Center for Mitochondrial and Epigenomic Medicine at the Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Yadava N; Pioneer Valley Life Sciences Institute, Springfield, MA 01199, USA; Division of Endocrinology, Diabetes & Metabolism at Baystate Medical Center, Tufts University School of Medicine, Springfield, MA 01199, USA. Electronic address: Nagendra.Yadava@baystatehealth.org.

Neurochem Int ; 109: 78-93, 2017 Oct.

Article em En | MEDLINE | ID: mdl-28506826

Assuntos

Complexo I de Transporte de Elétrons/deficiência; Complexo I de Transporte de Elétrons/genética; Genes Ligados ao Cromossomo X/genética; Proteínas de Membrana/genética; Doenças Neurodegenerativas/genética; Doenças Neurodegenerativas/metabolismo; Animais; Temperatura Corporal/fisiologia; Expiração/fisiologia; Feminino; Predisposição Genética para Doença/genética; Masculino; Camundongos; Camundongos da Linhagem 129; Camundongos Transgênicos; Gravidez

Palavras-chave

Complex I-Deficient mouse model; MWFE; Mitochondrial disease; NADH-Ubiquinone oxidoreductase; NDUFA1; Respiratory chain complex I

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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Doenças Neurodegenerativas / Complexo I de Transporte de Elétrons / Genes Ligados ao Cromossomo X / Proteínas de Membrana Idioma: En Ano de publicação: 2017 Tipo de documento: Article

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