SMN Deficiency Destabilizes ABCA1 Expression in Human Fibroblasts: Novel Insights in Pathophysiology of Spinal Muscular Atrophy.

Gabanella, Francesca; Onori, Annalisa; Pisani, Cinzia; Fiore, Marco; Ferraguti, Giampiero; Colizza, Andrea; de Vincentiis, Marco; Ceccanti, Marco; Inghilleri, Maurizio; Corbi, Nicoletta; Passananti, Claudio; Di Certo, Maria Grazia

Gabanella, Francesca; Onori, Annalisa; Pisani, Cinzia; Fiore, Marco; Ferraguti, Giampiero; Colizza, Andrea; de Vincentiis, Marco; Ceccanti, Marco; Inghilleri, Maurizio; Corbi, Nicoletta; Passananti, Claudio; Di Certo, Maria Grazia.

Afiliação

Gabanella F; CNR-Institute of Biochemistry and Cell Biology, Department of Sense Organs, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy.
Onori A; CNR-Institute of Molecular Biology and Pathology, Department of Molecular Medicine, Sapienza University of Rome, Viale Regina Elena 291, 00161 Rome, Italy.
Pisani C; CNR-Institute of Molecular Biology and Pathology, Department of Molecular Medicine, Sapienza University of Rome, Viale Regina Elena 291, 00161 Rome, Italy.
Fiore M; CNR-Institute of Biochemistry and Cell Biology, Department of Sense Organs, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy.
Ferraguti G; Department of Experimental Medicine, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy.
Colizza A; Department of Sense Organs, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy.
de Vincentiis M; Department of Sense Organs, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy.
Ceccanti M; Center for Rare Neuromuscular Diseases, Department of Human Neuroscience, Policlinico Umberto I, Sapienza University of Rome, 00185 Rome, Italy.
Inghilleri M; Center for Rare Neuromuscular Diseases, Department of Human Neuroscience, Policlinico Umberto I, Sapienza University of Rome, 00185 Rome, Italy.
Corbi N; CNR-Institute of Molecular Biology and Pathology, Department of Molecular Medicine, Sapienza University of Rome, Viale Regina Elena 291, 00161 Rome, Italy.
Passananti C; CNR-Institute of Molecular Biology and Pathology, Department of Molecular Medicine, Sapienza University of Rome, Viale Regina Elena 291, 00161 Rome, Italy.
Di Certo MG; CNR-Institute of Biochemistry and Cell Biology, Department of Sense Organs, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy.

Int J Mol Sci ; 24(3)2023 Feb 02.

Article em En | MEDLINE | ID: mdl-36769246

Assuntos

Neurônios Motores; Atrofia Muscular Espinal; Humanos; Neurônios Motores/metabolismo; Atrofia Muscular Espinal/metabolismo; Fatores de Transcrição/metabolismo; Fibroblastos/metabolismo; Proteínas de Membrana/metabolismo; Proteína 1 de Sobrevivência do Neurônio Motor/genética; Proteína 1 de Sobrevivência do Neurônio Motor/metabolismo; Transportador 1 de Cassete de Ligação de ATP/genética; Transportador 1 de Cassete de Ligação de ATP/metabolismo

Palavras-chave

ABCA1; SMA; SMN; cholesterol; plasma membrane

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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Atrofia Muscular Espinal / Neurônios Motores Limite: Humans Idioma: En Revista: Int J Mol Sci Ano de publicação: 2023 Tipo de documento: Article País de afiliação: Itália

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