Genomics

Dataset Information

44

Myostatin inhibition prevents skeletal muscle pathophysiology in Huntington’s disease mice.


ABSTRACT: Huntington’s disease (HD) is an inherited neurodegenerative disorder of which skeletal muscle atrophy is a common feature, and multiple lines of evidence support a muscle-based pathophysiology in HD mouse models. Inhibition of myostatin signaling increases muscle mass, and therapeutic approaches based on this are in clinical development. We have used a soluble ActRIIB decoy receptor (ACVR2B/Fc) to test the effects of myostatin/activin A inhibition in the R6/2 mouse model of HD. Transcriptional profiling of muscle in treated and untreated wild-type and R6/2 mice was performed to analyze the effect of the ActRIIB decoy on genes and pathways involved in maintaining normal muscle physiology as well as those dysregulated due to the mutant HTT gene mutation. Overall design: RNAseq was performed on tibialis muscle from wild-type, wildtype + decoy, R6/2 and R6/2 + decoy; N = 10 per group. RNAseq was done on an Illumina Hi-seq 2000. Paired-end sequencing was obtained, 4-plexed across lanes for a minimum of 38 million 50mer paired reads per sample

INSTRUMENT(S): Illumina HiSeq 2000 (Mus musculus)

SUBMITTER: Jeff Aaronson  

PROVIDER: GSE81367 | GEO | 2017-10-29

SECONDARY ACCESSION(S): PRJNA321381

REPOSITORIES: GEO

Dataset's files

Source:
Action DRS
GSE81367_ActRIIB_FPKM.xlsx.gz Xlsx
GSE81367_ActRIIB_counts.xlsx.gz Xlsx
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Publications


Huntington's disease (HD) is an inherited neurodegenerative disorder of which skeletal muscle atrophy is a common feature, and multiple lines of evidence support a muscle-based pathophysiology in HD mouse models. Inhibition of myostatin signaling increases muscle mass, and therapeutic approaches based on this are in clinical development. We have used a soluble ActRIIB decoy receptor (ACVR2B/Fc) to test the effects of myostatin/activin A inhibition in the R6/2 mouse model of HD. Weekly administra  ...[more]

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