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Series GSE70213 Query DataSets for GSE70213
Status Public on Jun 25, 2015
Title The effect of Nebulin-Deficiency on Skeletal Muscle
Organism Mus musculus
Experiment type Expression profiling by array
Summary Nebulin is a giant filamentous protein that is coextensive with the actin filaments of the skeletal muscle sarcomere. Nebulin mutations are the main cause of nemaline myopathy (NEM), with typical NEM adult patients having low expression of nebulin, yet the roles of nebulin in adult muscle remain poorly understood. To establish nebulin’s functional roles in adult muscle we performed studies on a novel conditional nebulin KO (Neb cKO) mouse model in which nebulin deletion was driven by the muscle creatine kinase (MCK) promotor. Neb cKO mice are born with high nebulin levels in their skeletal muscle but within weeks after birth nebulin expression rapidly falls to barely detectable levels Surprisingly, a large fraction of the mice survives to adulthood with low nebulin levels (<5% of control), contain nemaline rods, and undergo fiber-type switching towards oxidative types. These microarrays investigate the changes in gene expression when nebulin is deficient.
 
Overall design Two skeletal muscle groups were studied: Quadriceps (which is markedly smaller in the Neb cKO mice relative to control) and Soleus (which is not significantly smaller in the Neb cKO relative to control). Six biological replicates for each muscle group were selected; all are age-matched males.
 
Contributor(s) Granzier HL, Smith JE, Kolb J, Li F, Buck D
Citation(s) 26123491
Submission date Jun 24, 2015
Last update date Mar 04, 2019
Contact name Henk L Granzier
E-mail(s) granzier@email.arizona.edu
Organization name University of Arizona
Street address 1656 E Mabel St.
City Tucson
State/province AZ
ZIP/Postal code 85724
Country USA
 
Platforms (1)
GPL6246 [MoGene-1_0-st] Affymetrix Mouse Gene 1.0 ST Array [transcript (gene) version]
Samples (24)
GSM1720833 quad-control-1
GSM1720834 quad-control-2
GSM1720835 quad-control-3
Relations
BioProject PRJNA287882

Download family Format
SOFT formatted family file(s) SOFTHelp
MINiML formatted family file(s) MINiMLHelp
Series Matrix File(s) TXTHelp

Supplementary file Size Download File type/resource
GSE70213_RAW.tar 88.9 Mb (http)(custom) TAR (of CEL)
Raw data provided as supplementary file
Processed data included within Sample table

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