Coexpression of the CUG-Binding Protein Reduces DM Protein Kinase Expression in COS Cells
In: Journal of Biochemistry, Jg. 130 (2001-11-01), S. 581-587
Online
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Zugriff:
Myotonic dystrophy (DM) is the most common form of adult onset muscular dystrophy. Patients have a large CTG repeat expansion in the 3' untranslated region of the DMPK gene, which encodes DM protein kinase. RNA trans-dominant models, which hypothesize that the expanded CUG trinucleotide repeat on DMPK mRNA sequesters a factor or disrupts the RNA metabolism of the DMPK mRNA itself and other mRNAs in a trans dominant manner, have been proposed. A candidate for the sequestered factor, termed CUG-binding protein (CUG-BP), exists in several alternatively spliced isoforms. We found a human isoform with a twelve base insertion (deduced amino acids Leu-Tyr-Leu-Gln) and an isoform with a three base insertion (deduced amino acid Ala) insertion. In order to elucidate the effects of CUG-BP on DMPK expression, we introduced CUG-BP and DMPK cDNA transiently into COS-7 cells. Cotransfection of CUG-BP did not significantly affect the expression of either wild type or mutant DMPK at the mRNA level. On the other hand, cotransfection of CUG-BP significantly affected the expression of both the wild type and mutant DMPKs at the protein level. This reduction was remarkable when the mutant DMPK construct was used.
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Coexpression of the CUG-Binding Protein Reduces DM Protein Kinase Expression in COS Cells
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Autor/in / Beteiligte Person: | Usuki, Fusako ; Kino, Yosbihiro ; Ishiura, Shoichi ; Suzuki, Koichi ; Sorimachi, Hiroyuki ; Kawahara, Hiroyuki ; Sasagawa, Noboru ; Maeda, Tatsuya ; Takahashi, Nobuhiro |
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Zeitschrift: | Journal of Biochemistry, Jg. 130 (2001-11-01), S. 581-587 |
Veröffentlichung: | Oxford University Press (OUP), 2001 |
Medientyp: | unknown |
ISSN: | 0021-924X (print) |
DOI: | 10.1093/oxfordjournals.jbchem.a003022 |
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