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ArtikelCREB-Binding Protein Modulates Repeat Instability in a Drosophila Model for PolyQ Disease  
Oleh: Jung, Joonil ; Bonini, Nancy
Jenis: Article from Bulletin/Magazine
Dalam koleksi: SCIENCE (keterangan: ada di Proquest) vol. 315 no. 5820 (Mar. 2007), page 1857.
Ketersediaan
  • Perpustakaan FK
    • Nomor Panggil: S01.K.2007.03
    • Non-tandon: 1 (dapat dipinjam: 0)
    • Tandon: tidak ada
    Lihat Detail Induk
Isi artikelAlthough expansion of trinucleotide repeats accounts for over 30 human diseases, mechanisms of repeat instability remain poorly understood. We show that a Drosophila model for the CAGI polyglutamine (polyQ) disease spinocerebellar ataxia type 3 recapitulates key features of human CAG-repeat instability, including large repeat changes and strong expansion bias. Instability is dramatically enhanced by transcription and modulated by nuclear excision repair and a regulator of DNA repair adenosine 3',5'-monophosphate (cAMP) response element-binding protein (CREB)binding protein-a histone acetyltransferase (HAT) whose decreased activity contributes to polyQ disease. Pharmacological treatment to normalize acetylation suppressed instability. Thus, toxic consequences of pathogenic polyQ protein may include enhancing repeat instability.
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