Distinct conformers of transmissible misfolded SOD1 distinguish human SOD1-FALS from other forms of familial and sporadic ALS.

Ayers, Jacob I; Diamond, Jeffrey; Sari, Adriana; Fromholt, Susan; Galaleldeen, Ahmad; Ostrow, Lyle W; Glass, Jonathan D; Hart, P John; Borchelt, David R

Ayers, Jacob I; Diamond, Jeffrey; Sari, Adriana; Fromholt, Susan; Galaleldeen, Ahmad; Ostrow, Lyle W; Glass, Jonathan D; Hart, P John; Borchelt, David R.

Afiliação

Ayers JI; Department of Neuroscience, Center for Translational Research in Neurodegenerative Disease, University of Florida, Box 100159, Gainesville, FL, 32610, USA. jayers.123ja@ufl.edu.
Diamond J; Department of Neuroscience, Center for Translational Research in Neurodegenerative Disease, University of Florida, Box 100159, Gainesville, FL, 32610, USA.
Sari A; Department of Neuroscience, Center for Translational Research in Neurodegenerative Disease, University of Florida, Box 100159, Gainesville, FL, 32610, USA.
Fromholt S; Department of Neuroscience, Center for Translational Research in Neurodegenerative Disease, University of Florida, Box 100159, Gainesville, FL, 32610, USA.
Galaleldeen A; Department of Biological Sciences, St. Mary's University, San Antonio, TX, 78228, USA.
Ostrow LW; Department of Biochemistry and X-ray Crystallography Core Laboratory, University of Texas Health Science Center, San Antonio, TX, 78229, USA.
Glass JD; Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, 21205, USA.
Hart PJ; Department of Neurology, Emory University School of Medicine, Atlanta, GA, 30322, USA.
Borchelt DR; Department of Biological Sciences, St. Mary's University, San Antonio, TX, 78228, USA.

Acta Neuropathol ; 132(6): 827-840, 2016 12.

Article em En | MEDLINE | ID: mdl-27704280

Assuntos

Esclerose Lateral Amiotrófica/genética; Superóxido Dismutase-1/genética; Amiloide/genética; Amiloide/metabolismo; Esclerose Lateral Amiotrófica/diagnóstico; Esclerose Lateral Amiotrófica/patologia; Análise de Variância; Animais; Proteínas de Bactérias/genética; Proteínas de Bactérias/metabolismo; Modelos Animais de Doenças; Humanos; Técnicas In Vitro; Proteínas Luminescentes/genética; Proteínas Luminescentes/metabolismo; Camundongos; Camundongos Transgênicos; Microscopia Eletrônica; Mutação/genética; Técnicas de Cultura de Órgãos; Dobramento de Proteína; Deficiências na Proteostase/diagnóstico; Deficiências na Proteostase/genética; Medula Espinal/metabolismo; Medula Espinal/patologia; Medula Espinal/ultraestrutura; Superóxido Dismutase-1/metabolismo

Palavras-chave

Amyotrophic lateral sclerosis; Prion; Strains; Superoxide dismutase-1; fALS; sALS

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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Superóxido Dismutase-1 / Esclerose Lateral Amiotrófica Tipo de estudo: Diagnostic_studies / Prognostic_studies Limite: Animals / Humans Idioma: En Ano de publicação: 2016 Tipo de documento: Article

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