Impaired ribosome-associated quality control of C9orf72 arginine-rich dipeptide-repeat proteins.

Viera Ortiz, Ashley P; Cajka, Gregory; Olatunji, Olamide A; Mikytuck, Bailey; Shalem, Ophir; Lee, Edward B

Viera Ortiz, Ashley P; Cajka, Gregory; Olatunji, Olamide A; Mikytuck, Bailey; Shalem, Ophir; Lee, Edward B.

Afiliação

Viera Ortiz AP; Biochemistry and Molecular Biophysics Graduate Group, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA.
Cajka G; Translational Neuropathology Research Laboratory, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA.
Olatunji OA; Center for Cellular and Molecular Therapeutics, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Mikytuck B; Department of Genetics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA.
Shalem O; Translational Neuropathology Research Laboratory, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA.
Lee EB; Translational Neuropathology Research Laboratory, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA.

Brain ; 146(7): 2897-2912, 2023 07 03.

Article em En | MEDLINE | ID: mdl-36516294

Assuntos

Esclerose Lateral Amiotrófica; Demência Frontotemporal; Doenças Neurodegenerativas; Animais; Humanos; Dipeptídeos/genética; Proteína C9orf72/genética; Proteína C9orf72/metabolismo; Esclerose Lateral Amiotrófica/metabolismo; Doenças Neurodegenerativas/genética; Ribossomos; Expansão das Repetições de DNA/genética; Demência Frontotemporal/genética; Mamíferos/genética; Mamíferos/metabolismo

Palavras-chave

C9orf72; ALS/FTD; NEMF; RAN translation; ribosome-associated quality control

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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Doenças Neurodegenerativas / Demência Frontotemporal / Esclerose Lateral Amiotrófica Tipo de estudo: Prognostic_studies / Risk_factors_studies Limite: Animals / Humans Idioma: En Ano de publicação: 2023 Tipo de documento: Article

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