Disruption of snRNP biogenesis factors Tgs1 and pICln induces phenotypes that mirror aspects of SMN-Gemins complex perturbation in Drosophila, providing new insights into spinal muscular atrophy.

Borg, Rebecca M; Fenech Salerno, Benji; Vassallo, Neville; Bordonne, Rémy; Cauchi, Ruben J

Borg, Rebecca M; Fenech Salerno, Benji; Vassallo, Neville; Bordonne, Rémy; Cauchi, Ruben J.

Afiliação

Borg RM; Department of Physiology and Biochemistry, Faculty of Medicine and Surgery, University of Malta, Msida, Malta; Centre for Molecular Medicine and Biobanking, Biomedical Sciences Building, University of Malta, Msida, Malta; Institut de Génétique Moléculaire de Montpellier, CNRS-UMR5535, Université Mon
Fenech Salerno B; Department of Physiology and Biochemistry, Faculty of Medicine and Surgery, University of Malta, Msida, Malta; Centre for Molecular Medicine and Biobanking, Biomedical Sciences Building, University of Malta, Msida, Malta.
Vassallo N; Department of Physiology and Biochemistry, Faculty of Medicine and Surgery, University of Malta, Msida, Malta; Centre for Molecular Medicine and Biobanking, Biomedical Sciences Building, University of Malta, Msida, Malta.
Bordonne R; Institut de Génétique Moléculaire de Montpellier, CNRS-UMR5535, Université Montpellier 1 and 2, Montpellier, France.
Cauchi RJ; Department of Physiology and Biochemistry, Faculty of Medicine and Surgery, University of Malta, Msida, Malta; Centre for Molecular Medicine and Biobanking, Biomedical Sciences Building, University of Malta, Msida, Malta. Electronic address: ruben.cauchi@um.edu.mt.

Neurobiol Dis ; 94: 245-58, 2016 Oct.

Article em En | MEDLINE | ID: mdl-27388936

Assuntos

Proteínas de Drosophila/metabolismo; Neurônios Motores/metabolismo; Ribonucleoproteínas Nucleares Pequenas/metabolismo; Proteínas do Complexo SMN/metabolismo; Animais; Citoplasma/metabolismo; Proteínas de Drosophila/genética; Drosophila melanogaster; Fenótipo

Palavras-chave

Gemin2; Gemin3; SMN complex; Strap; Tgs1; Unrip; motor neuron disease; pICln; snRNP assembly; spinal muscular atrophy; trimethylguanosine synthase 1; wmd

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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Ribonucleoproteínas Nucleares Pequenas / Proteínas de Drosophila / Proteínas do Complexo SMN / Neurônios Motores Limite: Animals Idioma: En Revista: Neurobiol Dis Assunto da revista: NEUROLOGIA Ano de publicação: 2016 Tipo de documento: Article

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