iASPP, a previously unidentified regulator of desmosomes, prevents arrhythmogenic right ventricular cardiomyopathy (ARVC)-induced sudden death.

Notari, Mario; Hu, Ying; Sutendra, Gopinath; Dedeic, Zinaida; Lu, Min; Dupays, Laurent; Yavari, Arash; Carr, Carolyn A; Zhong, Shan; Opel, Aaisha; Tinker, Andrew; Clarke, Kieran; Watkins, Hugh; Ferguson, David J P; Kelsell, David P; de Noronha, Sofia; Sheppard, Mary N; Hollinshead, Mike; Mohun, Timothy J; Lu, Xin

Notari, Mario; Hu, Ying; Sutendra, Gopinath; Dedeic, Zinaida; Lu, Min; Dupays, Laurent; Yavari, Arash; Carr, Carolyn A; Zhong, Shan; Opel, Aaisha; Tinker, Andrew; Clarke, Kieran; Watkins, Hugh; Ferguson, David J P; Kelsell, David P; de Noronha, Sofia; Sheppard, Mary N; Hollinshead, Mike; Mohun, Timothy J; Lu, Xin.

Afiliação

Notari M; Ludwig Institute for Cancer Research Ltd., Nuffield Department of Clinical Medicine, University of Oxford, Oxford OX3 7DQ, United Kingdom;
Hu Y; Ludwig Institute for Cancer Research Ltd., Nuffield Department of Clinical Medicine, University of Oxford, Oxford OX3 7DQ, United Kingdom;
Sutendra G; Ludwig Institute for Cancer Research Ltd., Nuffield Department of Clinical Medicine, University of Oxford, Oxford OX3 7DQ, United Kingdom;
Dedeic Z; Ludwig Institute for Cancer Research Ltd., Nuffield Department of Clinical Medicine, University of Oxford, Oxford OX3 7DQ, United Kingdom;
Lu M; Ludwig Institute for Cancer Research Ltd., Nuffield Department of Clinical Medicine, University of Oxford, Oxford OX3 7DQ, United Kingdom;
Dupays L; Developmental Biology Division, Medical Research Council National Institute for Medical Research, London NW7 1AA, United Kingdom;
Yavari A; Division of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, Oxford OX3 9DU, United Kingdom; Wellcome Trust Centre for Human Genetics, University of Oxford, Oxford OX3 7BN, United Kingdom;
Carr CA; Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford OX1 3PT, United Kingdom;
Zhong S; Ludwig Institute for Cancer Research Ltd., Nuffield Department of Clinical Medicine, University of Oxford, Oxford OX3 7DQ, United Kingdom;
Opel A; Department of Medicine, University College London, London WC1E 6JJ, United Kingdom;
Tinker A; Department of Medicine, University College London, London WC1E 6JJ, United Kingdom;
Clarke K; Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford OX1 3PT, United Kingdom;
Watkins H; Division of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, Oxford OX3 9DU, United Kingdom; Wellcome Trust Centre for Human Genetics, University of Oxford, Oxford OX3 7BN, United Kingdom;
Ferguson DJ; Nuffield Department of Clinical Laboratory Science, University of Oxford, John Radcliffe Hospital, Oxford, OX3 9DU, United Kingdom;
Kelsell DP; Centre for Cutaneous Research, The Blizard Institute, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, London E1 2AT, United Kingdom;
de Noronha S; Centre for Cardiovascular Risk in Younger Persons, Department of Cardiovascular Pathology, Cardiovascular Sciences Research Centre, St. George's University of London, London SW17 0RE, United Kingdom; and.
Sheppard MN; Centre for Cardiovascular Risk in Younger Persons, Department of Cardiovascular Pathology, Cardiovascular Sciences Research Centre, St. George's University of London, London SW17 0RE, United Kingdom; and.
Hollinshead M; Section of Virology, Faculty of Medicine, Imperial College London, London W2 1PG, United Kingdom.
Mohun TJ; Developmental Biology Division, Medical Research Council National Institute for Medical Research, London NW7 1AA, United Kingdom;
Lu X; Ludwig Institute for Cancer Research Ltd., Nuffield Department of Clinical Medicine, University of Oxford, Oxford OX3 7DQ, United Kingdom; Xin.Lu@ludwig.ox.ac.uk.

Proc Natl Acad Sci U S A ; 112(9): E973-81, 2015 Mar 03.

Article em En | MEDLINE | ID: mdl-25691752

Assuntos

Arritmias Cardíacas; Cardiomiopatia Hipertrófica Familiar; Morte Súbita; Desmossomos; Peptídeos e Proteínas de Sinalização Intracelular; Proteínas Repressoras; Substituição de Aminoácidos; Animais; Arritmias Cardíacas/genética; Arritmias Cardíacas/metabolismo; Arritmias Cardíacas/patologia; Sequência de Bases; Cardiomiopatia Hipertrófica Familiar/genética; Cardiomiopatia Hipertrófica Familiar/metabolismo; Cardiomiopatia Hipertrófica Familiar/patologia; Bovinos; Linhagem Celular Transformada; Desmina/genética; Desmina/metabolismo; Desmoplaquinas/genética; Desmoplaquinas/metabolismo; Desmossomos/genética; Desmossomos/metabolismo; Desmossomos/patologia; Modelos Animais de Doenças; Feminino; Humanos; Filamentos Intermediários; Peptídeos e Proteínas de Sinalização Intracelular/genética; Peptídeos e Proteínas de Sinalização Intracelular/metabolismo; Masculino; Camundongos; Mutação de Sentido Incorreto; Proteínas Repressoras/genética; Proteínas Repressoras/metabolismo; Deleção de Sequência

Palavras-chave

ARVC; cellcell junctions; desmosome; iASPP; sudden death

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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Arritmias Cardíacas / Proteínas Repressoras / Cardiomiopatia Hipertrófica Familiar / Morte Súbita / Peptídeos e Proteínas de Sinalização Intracelular / Desmossomos Tipo de estudo: Prognostic_studies Limite: Animals / Female / Humans / Male Idioma: En Revista: Proc Natl Acad Sci U S A Ano de publicação: 2015 Tipo de documento: Article

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