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- Title
Coexpression of the CUG-Binding Protein Reduces DM Protein Kinase Expression in COS Cells1.
- Authors
Takahashi, Nobuhiro; Sasagawa, Noboru; Usuki, Fusako; Kino, Yosbihiro; Kawahara, Hiroyuki; Sorimachi, Hiroyuki; Maeda, Tatsuya; Suzuki, Koichi; Ishiura, Shoichi
- Abstract
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 fra/is-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 isofonns. 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 DMPK at the protein level. This reduction was remarkable when the mutant DMPK construct was used.
- Subjects
MYOTONIA atrophica; MUSCULAR dystrophy; PROTEIN kinases; CARRIER proteins; MESSENGER RNA
- Publication
Journal of Biochemistry, 2001, Vol 130, Issue 5, p581
- ISSN
0021-924X
- Publication type
Article
- DOI
10.1093/oxfordjournals.jbchem.a003022