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Links from GEO DataSets

Items: 10

1.
Full record GDS5880

Nebulin deficiency effect on the quadriceps

Analysis of quadriceps muscles from 6 week old males with a conditional knockout of nebulin in their striated muscles. Nebulin is a giant filamentous protein that is coextensive with actin filaments of the skeletal muscle sarcomere. Results provide insight into the role of nebulin in adult muscles.
Organism:
Mus musculus
Type:
Expression profiling by array, count, 2 genotype/variation sets
Platform:
GPL6246
Series:
GSE70213
12 Samples
Download data: CEL
2.

The effect of Nebulin-Deficiency on Skeletal Muscle

(Submitter supplied) 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. more...
Organism:
Mus musculus
Type:
Expression profiling by array
Datasets:
GDS5880 GDS5881
Platform:
GPL6246
24 Samples
Download data: CEL
Series
Accession:
GSE70213
ID:
200070213
3.
Full record GDS5881

Nebulin deficiency effect on the soleus

Analysis of soleus muscles from 6 week old males with a conditional knockout of nebulin in their striated muscles. Nebulin is a giant filamentous protein that is coextensive with actin filaments of the skeletal muscle sarcomere. Results provide insight into the role of nebulin in adult muscles.
Organism:
Mus musculus
Type:
Expression profiling by array, count, 2 genotype/variation sets
Platform:
GPL6246
Series:
GSE70213
12 Samples
Download data: CEL
4.

Comparison of genes regulated in P0 Klhl40 WT and KO skeletal muscle

(Submitter supplied) Nemaline myopathy (NM) is a congenital myopathy that can result in lethal muscle dysfunction and is thought to be a disease of the sarcomere thin filament. Recently, several proteins of unknown function have been implicated in NM, and their role in the disease remains unresolved. Here, we demonstrate that loss of a muscle-specific protein, Klhl40, results in a nemaline-like myopathy in mice that closely phenocopies the muscle abnormalities observed KLHL40 deficient patients. more...
Organism:
Mus musculus
Type:
Expression profiling by array
Dataset:
GDS5295
Platform:
GPL6887
6 Samples
Download data: TXT
Series
Accession:
GSE56570
ID:
200056570
5.
Full record GDS5295

Kelch-like family member 40 deficiency effect on the skeletal muscle

Analysis of quadriceps muscles of mutants lacking kelch-like family member 40 (KLHL40). KLHL40 mutations occur in severe nemaline myopathies (NMs). NMs are congenital myopathies that can result in lethal muscle dysfunction. Results provide insight into the role of KLHL40 in the pathogenesis of NM.
Organism:
Mus musculus
Type:
Expression profiling by array, count, 2 genotype/variation sets
Platform:
GPL6887
Series:
GSE56570
6 Samples
Download data
6.

Genome-wide gene expression analysis on tibialis anterior muscle from nebulin SH3 domain deleted (Neb∆SH3) mice

(Submitter supplied) Genome-wide gene expression analysis on tibialis anterior muscle from 2-month-old nebulin SH3 domain deleted (Neb∆SH3) mice compared to wildtype.
Organism:
Mus musculus
Type:
Expression profiling by array
Platform:
GPL6887
6 Samples
Download data: TXT
Series
Accession:
GSE47801
ID:
200047801
7.

KLHL41 stabilizes skeletal muscle sarcomeres by nonproteolytic ubiquitination

(Submitter supplied) Maintenance of muscle function requires assembly of contractile proteins into highly organized sarcomeres. Mutations in Kelch-like protein 41 (KLHL41) cause nemaline myopathy, a fatal muscle disorder associated with sarcomere disarray. We generated KLHL41 mutant mice, which display lethal disruption of sarcomeres and aberrant expression of muscle structural and contractile proteins, mimicking the hallmarks of the human disease. more...
Organism:
Mus musculus
Type:
Expression profiling by high throughput sequencing
Platform:
GPL17021
6 Samples
Download data: TXT
Series
Accession:
GSE95543
ID:
200095543
8.

Nemaline myopathy mouse model

(Submitter supplied) The aim of this study was to investigate the molecular mechanisms implicated in this mouse model of nemaline myopathy, and to further compare the molecular disease response in different skeletal muscles. For this purpose, snap frozen skeletla muscle specimens from wild type and transgenic for alpha tropomyosin slow mice were studied. Five different muscle types were used (diaphragm, plantaris, extensor digitorum longus, tibialis anterior, gastrocnemus). more...
Organism:
Mus musculus
Type:
Expression profiling by array
Dataset:
GDS2312
Platform:
GPL81
36 Samples
Download data: CEL, EXP
Series
Accession:
GSE3384
ID:
200003384
9.
Full record GDS2312

Nemaline myopathy model: various skeletal muscles

Analysis of various skeletal muscles of trangenics expressing the human Met9Arg alpha-tropomyosin slow mutant gene, a model of nemaline myopathy (NM). NM, a non-dystrophic congenital myopathy, is a variably severe neuromuscular disorder. Results provide insight into the pathogenesis of NM.
Organism:
Mus musculus
Type:
Expression profiling by array, count, 2 age, 2 disease state, 5 tissue sets
Platform:
GPL81
Series:
GSE3384
36 Samples
Download data: CEL, EXP
10.

Gene expression profiling of NUAK kinase overexpression in Drosophila larval muscle development

(Submitter supplied) Signal transduction pathways mediated by kinases control diverse biological outputs at the level of cells and tissues to regulate a diverse array of biological and developmental events. To gain insight into how muscle expression of the evolutionarily conserved NUAK kinase regulates the transcriptional landscape during Drosophila melanogaster development, we performed high-throughput sequencing of RNA from either whole larvae or dissected muscle fillets at the end of larval development. more...
Organism:
Drosophila melanogaster
Type:
Expression profiling by high throughput sequencing
Platform:
GPL21306
18 Samples
Download data: TXT
Series
Accession:
GSE204894
ID:
200204894
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