Regulation of the promoter of CUG triplet repeat binding protein, Cugbp1, during myogenesis.

Regulation of the promoter of CUG triplet repeat binding protein, Cugbp1, during myogenesis. Research Abstract Details 

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Regulation of the promoter of CUG triplet repeat binding protein, Cugbp1, during myogenesis. Abstract Text:

Claudia H Huichalaf,Keiko Sakai,Guo-Li Wang,Nikolai A Timchenko,Lubov Timchenko,

CUG triplet repeat binding protein, CUGBP1, plays a critical role in the development of skeletal muscle pathology in patients with Myotonic Dystrophy 1 (DM1). In this paper, we have characterized transcriptional regulation of mouse Cugbp1 gene during normal myogenesis. There are several Cugbp1 mRNA species with variable 5' ends. We found that these mRNA species have different patterns of expression during myogenesis. Isoforms 1 and 2 are mainly expressed in myotubes, while expression of isoform 3 is increased during transition of myoblasts to myotubes and during transition of myotubes to myofibers. We have cloned a short region of the Cugbp1 promoter, which is responsible for the regulation of the isoform 3, and have identified within this region three different transcription start sites. This promoter region exhibits high activity in myoblasts and the activity of this region is significantly increased in myotubes. The Cugbp1 promoter contains three E-box elements. A mutation of one of the E-boxes, E(3), significantly reduces activity of the Cugbp1 promoter. Gelshift and ChIP assays showed that E(3)-box is occupied by E12, CBP and p300 proteins in myoblasts, while in differentiated myotubes this element is occupied by myogenin, E12 and p300. The binding of myogenin to the Cugbp1 promoter correlates with activation of the promoter during differentiation. Our data show that myogenin is a key regulator of the Cugbp1 promoter since overexpression of myogenin increases the activity of the Cugbp1 promoter; while the inhibition of myogenin reduces activity of the Cugbp1 promoter. These data show that transcription of Cugbp1 gene in muscle is regulated by myogenin and E proteins and suggest that the co-operation of several transcription factors is important for the activation of the Cugbp1 promoter.

Regulation of the promoter of CUG triplet repeat binding protein, Cugbp1, during myogenesis. Publishing Authors By Initials

CH Huichalaf,K Sakai,GL Wang,NA Timchenko,L Timchenko,

For similar biochemical phenomena, metabolism, and nutrition: biochemical phenomena: molecular structure: base sequence: repetitive sequences, nucleic acid: tandem repeat sequences: microsatellite repeats: trinucleotide repeats research abstracts see: biochemical phenomena, metabolism, and nutrition: biochemical phenomena: molecular structure: base sequence: repetitive sequences, nucleic acid: tandem repeat sequences: microsatellite repeats: trinucleotide repeats research

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Regulation of the promoter of CUG triplet repeat binding protein, Cugbp1, during myogenesis. Journal Published:

PUBLICATION TYPE: Research Support, N.I.H., Extr

Journal: Gene

VOLUME: 396

Page Numbers: 391-402

Journal Abbreviation: Gene

ISSN: 0378-1119

DAY: 29

MONTH: 04

YEAR: 2007

Regulation of the promoter of CUG triplet repeat binding protein, Cugbp1, during myogenesis. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 7706761

Regulation of the promoter of CUG triplet repeat binding protein, Cugbp1, during myogenesis. Keywords Mesh Terms:

KEYWORDS: Trinucleotide Repeats

MESH TERMS: physiology

Chemical & Substance for Abstract: Regulation of the promoter of CUG triplet repeat binding protein, Cugbp1, during myogenesis. Information

Substance Name: RNA-Binding Proteins

Registry Number: 0

Grant and Affiliation Information for Regulation of the promoter of CUG triplet repeat binding protein, Cugbp1, during myogenesis.

AFFILIATION: Department of Medicine, Section of Cardiovascular Sciences, Baylor College of Medicine, One Baylor Plaza, Houston Texas 77030, USA.

Country: Netherlands

AGENCY: United States NIGMS

GRANT: GM55188

ACRONYM: GM

MEDLINETA: Gene

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