دورية أكاديمية

Nucleolar localization of aprataxin is dependent on interaction with nucleolin and on active ribosomal DNA transcription

التفاصيل البيبلوغرافية
العنوان: Nucleolar localization of aprataxin is dependent on interaction with nucleolin and on active ribosomal DNA transcription
المؤلفون: Becherel, Olivier J., Gueven, Nuri, Birrell, Geoff W., Schreiber, Val�rie, Suraweera, Amila, Jakob, Burkhard, Taucher-Scholz, Gisela, Lavin, Martin F.
المصدر: Human Molecular Genetics
بيانات النشر: Oxford University Press
سنة النشر: 2006
المجموعة: Queensland University of Technology: QUT ePrints
مصطلحات موضوعية: fibrinogen, signal transduction, nervous system disorder, mutation, cell nucleolus
الوصف: The APTX gene, mutated in patients with the neurological disorder ataxia with oculomotor apraxia type 1 (AOA1), encodes a novel protein aprataxin. We describe here, the interaction and interdependence between aprataxin and several nucleolar proteins, including nucleolin, nucleophosmin and upstream binding factor-1 (UBF-1), involved in ribosomal RNA (rRNA) synthesis and cellular stress signalling. Interaction between aprataxin and nucleolin occurred through their respective N-terminal regions. In AOA1 cells lacking aprataxin, the stability of nucleolin was significantly reduced. On the other hand, down-regulation of nucleolin by RNA interference did not affect aprataxin protein levels but abolished its nucleolar localization suggesting that the interaction with nucleolin is involved in its nucleolar targeting. GFP-aprataxin fusion protein co-localized with nucleolin, nucleophosmin and UBF-1 in nucleoli and inhibition of ribosomal DNA transcription altered the distribution of aprataxin in the nucleolus, suggesting that the nature of the nucleolar localization of aprataxin is also dependent on ongoing rRNA synthesis. In vivo rRNA synthesis analysis showed only a minor decrease in AOA1 cells when compared with controls cells. These results demonstrate a cross-dependence between aprataxin and nucleolin in the nucleolus and while aprataxin does not appear to be directly involved in rRNA synthesis its nucleolar localization is dependent on this synthesis.
نوع الوثيقة: article in journal/newspaper
اللغة: unknown
العلاقة: Becherel, Olivier J., Gueven, Nuri, Birrell, Geoff W., Schreiber, Val�rie, Suraweera, Amila, Jakob, Burkhard, Taucher-Scholz, Gisela, & Lavin, Martin F. (2006) Nucleolar localization of aprataxin is dependent on interaction with nucleolin and on active ribosomal DNA transcription. Human Molecular Genetics, 15(14), pp. 2239-2249.; https://eprints.qut.edu.au/133297Test/; Faculty of Health; School of Biomedical Sciences
الإتاحة: https://doi.org/10.1093/hmg/ddl149Test
https://eprints.qut.edu.au/133297Test/
رقم الانضمام: edsbas.C7F93EDE
قاعدة البيانات: BASE