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Series GSE106826 Query DataSets for GSE106826
Status Public on Mar 18, 2019
Title Loss of RNA-binding protein Sfpq causes long-gene transcriptopathy in skeletal muscle and severe muscle mass reduction with metabolic myopathy (Primary culture, rRNA depleted RNA-seq)
Organism Mus musculus
Experiment type Other
Summary Growing evidences are suggesting that extra-long genes in mammals are vulnerable for full-gene length transcription and dysregulation of long genes is a mechanism underlying human genetic disorders. Skeletal muscle expresses Dystrophin which is 2.26 Mbp in length; however, how long-distance transcription is achieved is totally unknown. We had discovered RNA-binding protein SFPQ preferentially binds to long pre-mRNAs and specifically regulates the cluster of neuronal genes > 100 kbp. Here we investigated the roles of SFPQ for long gene expression, target specificities, and also physiological functions in skeletal muscle. Loss of Sfpq selectively downregulated genes >100 kbp including Dystrophin and caused progressive muscle mass reduction and metabolic myopathy characterized by glycogen accumulation and decreased abundance of mitochondrial oxidative phosphorylation complexes. Functional clustering analysis identified metabolic pathway related genes as the targets of SFPQ. These findings indicate target gene specificities and tissue-specific physiological functions of SFPQ in skeletal muscle.
 
Overall design We analyzed rRNA-depleted RNA profiles including transcribing RNAs in primary myoblasts obtained from skeletal muscles of 1-month-old SfpqSM-KO (n=1) and control (n=1) mice under differentiated condition using Ion-proton.
 
Contributor(s) Hosokawa M, Takeuchi A, Iida K, Hagiwara M
Citation(s) 30870781
Submission date Nov 13, 2017
Last update date May 15, 2019
Contact name Kei IIDA
E-mail(s) kiida@life.kindai.ac.jp
Organization name Kindai University
Street address Kowakae 3-4-1
City Higashi-Osaka
State/province Osaka
ZIP/Postal code 5778502
Country Japan
 
Platforms (1)
GPL18635 Ion Torrent Proton (Mus musculus)
Samples (2)
GSM2854982 PrimaryMyoblast__Cont__rRNAdep.rep1
GSM2854983 PrimaryMyoblast__SFPQ_KO__rRNAdep.rep1
This SubSeries is part of SuperSeries:
GSE106827 Loss of RNA-binding protein Sfpq causes long-gene transcriptopathy in skeletal muscle and severe muscle mass reduction with metabolic myopathy
Relations
BioProject PRJNA418145
SRA SRP124852

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Supplementary file Size Download File type/resource
GSE106826_RAW.tar 211.2 Mb (http)(custom) TAR (of BEDGRAPH)
SRA Run SelectorHelp
Raw data are available in SRA
Processed data provided as supplementary file

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