Culture Conditions Affect Expression of DUX4 in FSHD Myoblasts
نویسندگان
چکیده
منابع مشابه
Culture Conditions Affect Expression of DUX4 in FSHD Myoblasts.
Facioscapulohumeral muscular dystrophy (FSHD) is believed to be caused by aberrant expression of double homeobox 4 (DUX4) due to epigenetic changes of the D4Z4 region at chromosome 4q35. Detecting DUX4 is challenging due to its stochastic expression pattern and low transcription level. In this study, we examined different cDNA synthesis strategies and the sensitivity for DUX4 detection. In addi...
متن کاملPARP1 Differentially Interacts with Promoter region of DUX4 Gene in FSHD Myoblasts
OBJECTIVE The goal of the study is to identity proteins, which interact with the promoter region of double homeobox protein 4 (DUX4) gene known to be causative for the autosomal dominant disorder Facioscapulohumeral Muscular Dystrophy (FSHD). METHODS We performed a DNA pull down assay coupled with mass spectrometry analysis to identify proteins that interact with a DUX4 promoter probe in Rhab...
متن کاملThe FSHD Atrophic Myotube Phenotype Is Caused by DUX4 Expression
BACKGROUND Facioscapulohumeral muscular dystrophy (FSHD) is linked to deletions in 4q35 within the D4Z4 repeat array in which we identified the double homeobox 4 (DUX4) gene. We found stable DUX4 mRNAs only derived from the most distal D4Z4 unit and unexpectedly extended to the flanking pLAM region that provided an intron and a polyadenylation signal. DUX4 encodes a transcription factor express...
متن کاملModel systems of DUX4 expression recapitulate the transcriptional profile of FSHD cells.
Facioscapulohumeral dystrophy (FSHD) is caused by the mis-expression of the double-homeodomain transcription factor DUX4 in skeletal muscle cells. Many different cell culture models have been developed to study the pathophysiology of FSHD, frequently based on endogenous expression of DUX4 in FSHD cells or by mis-expression of DUX4 in control human muscle cells. Although results generated using ...
متن کاملDUX4 and DUX4 downstream target genes are expressed in fetal FSHD muscles.
Facioscapulohumeral muscular dystrophy (FSHD) is one of the most prevalent adult muscular dystrophies. The common clinical signs usually appear during the second decade of life but when the first molecular dysregulations occur is still unknown. Our aim was to determine whether molecular dysregulations can be identified during FSHD fetal muscle development. We compared muscle biopsies derived fr...
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ژورنال
عنوان ژورنال: Molecules
سال: 2015
ISSN: 1420-3049
DOI: 10.3390/molecules20058304