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Titolo:
Visualization of double-stranded RNAs from the myotonic dystrophy protein kinase gene and interactions with CUG-binding protein
Autore:
Michalowski, S; Miller, JW; Urbinati, CR; Paliouras, M; Swanson, MS; Griffith, J;
Indirizzi:
Univ N Carolina, Lineberger Comprehens Canc Ctr, Chapel Hill, NC 27599 USAUniv N Carolina Chapel Hill NC USA 27599 c Ctr, Chapel Hill, NC 27599 USA Univ Florida, Coll Med, Dept Mol Genet & Microbiol, Gainesville, FL 32610 USA Univ Florida Gainesville FL USA 32610 icrobiol, Gainesville, FL 32610 USA Univ Florida, Coll Med, Ctr Gene Therapy, Gainesville, FL 32610 USA Univ Florida Gainesville FL USA 32610 Therapy, Gainesville, FL 32610 USA Univ Florida, Coll Med, Ctr Mammalian Genet, Gainesville, FL 32610 USA Univ Florida Gainesville FL USA 32610 an Genet, Gainesville, FL 32610 USA
Titolo Testata:
NUCLEIC ACIDS RESEARCH
fascicolo: 17, volume: 27, anno: 1999,
pagine: 3534 - 3542
SICI:
0305-1048(19990901)27:17<3534:VODRFT>2.0.ZU;2-5
Fonte:
ISI
Lingua:
ENG
Soggetto:
UNSTABLE CTG REPEAT; DNA TRIPLET REPEATS; TRINUCLEOTIDE REPEAT; MESSENGER-RNA; REDUCES EXPRESSION; MOLECULAR-BASIS; EXPANSION; TRANSCRIPTS; REGION; FORM;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
51
Recensione:
Indirizzi per estratti:
Indirizzo: Griffith, J Univ N Carolina, Lineberger Comprehens Canc Ctr, Chapel Hill, NC 27599 USA Univ N Carolina Chapel Hill NC USA 27599 l Hill, NC 27599 USA
Citazione:
S. Michalowski et al., "Visualization of double-stranded RNAs from the myotonic dystrophy protein kinase gene and interactions with CUG-binding protein", NUCL ACID R, 27(17), 1999, pp. 3534-3542

Abstract

Myotonic dystrophy (DM) is associated with a (CTG)(n) triplet repeat expansion in the 3'-untranslated region of the myotonic dystrophy protein kinase(DMPK) gene. Using electron microscopy, we visualized large RNAs containing up to 130 CUG repeats and studied the binding of purified CUG-binding protein (CUG-BP) to these RNAs, Electron microscopic examination revealed perfect double-stranded (ds)RNA segments whose lengths were that expected for duplex RNA, The RNA dominant mutation model for DM pathogenesis predicts that the expansion mutation acts at the RNA level by forming long dsRNAs that sequester certain RNA-binding proteins, To test this model, we examined thesubcellular distribution and RNA-binding properties of CUG-BP, While previous studies have demonstrated that mutant DMPK transcripts accumulate in nuclear foci, the localization pattern of CUG-BP in both normal and DM cells was similar. Although CUG-BP in nuclear extracts preferentially photocrosslinked to DMPK transcripts, this binding was not proportional to (CUG), repeat size. Moreover, CUG-BP localized to the base of the RNA hairpin and not along the stem, as visualized by electron microscopy, These results providethe first visual evidence that the DM expansion forms an RNA hairpin structure and suggest that CUG-BP is unlikely to be a sequestered factor.

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Documento generato il 12/07/20 alle ore 09:28:19