PMID- 11686919
OWN - NLM
STAT- MEDLINE
DCOM- 20020222
LR  - 20211203
IS  - 0021-924X (Print)
IS  - 0021-924X (Linking)
VI  - 130
IP  - 5
DP  - 2001 Nov
TI  - Coexpression of the CUG-binding protein reduces DM protein kinase expression in 
      COS cells.
PG  - 581-7
AB  - 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.
FAU - Takahashi, N
AU  - Takahashi N
AD  - Department of Life Sciences, Graduate School of Arts and Sciences, The University 
      of Tokyo, Komaba, Meguro-ku, Tokyo 153-8902, Japan.
FAU - Sasagawa, N
AU  - Sasagawa N
FAU - Usuki, F
AU  - Usuki F
FAU - Kino, Y
AU  - Kino Y
FAU - Kawahara, H
AU  - Kawahara H
FAU - Sorimachi, H
AU  - Sorimachi H
FAU - Maeda, T
AU  - Maeda T
FAU - Suzuki, K
AU  - Suzuki K
FAU - Ishiura, S
AU  - Ishiura S
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - J Biochem
JT  - Journal of biochemistry
JID - 0376600
RN  - 0 (CELF1 Protein)
RN  - 0 (CELF1 protein, human)
RN  - 0 (DMPK protein, human)
RN  - 0 (DMPK protein, mouse)
RN  - 0 (Protein Isoforms)
RN  - 0 (RNA, Messenger)
RN  - 0 (RNA-Binding Proteins)
RN  - 0 (Ribonucleoproteins)
RN  - 24937-83-5 (Poly A)
RN  - EC 2.7.11.1 (Myotonin-Protein Kinase)
RN  - EC 2.7.11.1 (Protein Serine-Threonine Kinases)
SB  - IM
MH  - Animals
MH  - Base Sequence
MH  - CELF1 Protein
MH  - COS Cells
MH  - Chlorocebus aethiops
MH  - Down-Regulation
MH  - *Gene Expression
MH  - Humans
MH  - Mice
MH  - Molecular Sequence Data
MH  - Mutation
MH  - Myotonic Dystrophy/genetics
MH  - Myotonin-Protein Kinase
MH  - Poly A/genetics
MH  - Protein Biosynthesis
MH  - Protein Isoforms
MH  - Protein Serine-Threonine Kinases/*biosynthesis/genetics
MH  - RNA, Messenger/analysis/biosynthesis
MH  - RNA-Binding Proteins/*biosynthesis/genetics
MH  - Ribonucleoproteins/*biosynthesis/genetics
MH  - Tissue Distribution
MH  - Trinucleotide Repeats/genetics
EDAT- 2001/11/01 10:00
MHDA- 2002/02/23 10:01
CRDT- 2001/11/01 10:00
PHST- 2001/11/01 10:00 [pubmed]
PHST- 2002/02/23 10:01 [medline]
PHST- 2001/11/01 10:00 [entrez]
AID - 10.1093/oxfordjournals.jbchem.a003022 [doi]
PST - ppublish
SO  - J Biochem. 2001 Nov;130(5):581-7. doi: 10.1093/oxfordjournals.jbchem.a003022.