Browse
Submit Data
Databases
API
Help

Metabolomics,Unknown,Transcriptomics,Genomics,Proteomics

Dataset Information

0 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Transcription profiling of skeletal muscle from wild type and Nebulin knock-out mice

ABSTRACT: Knock out of the Nebulin gene. We compared the Quadriceps of two Nebulin -deficient vs. 2 Wildtype mice. The two KO-WT pairs derived from two litters (F2 generation, background: C57/Bl6 and 129/IB10).

ORGANISM(S): Mus musculus

DISEASE(S): progressive skeletal muscle myopathy

SUBMITTER: Mark McNabb

PROVIDER: E-MEXP-733 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

ACCESS DATA

Json Xml

Publications

Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo.

Witt Christian C CC Burkart Christoph C Labeit Dietmar D McNabb Mark M Wu Yiming Y Granzier Henk H Labeit Siegfried S

The EMBO journal 20060810 16

The precise assembly of the highly organized filament systems found in muscle is critically important for its function. It has been hypothesized that nebulin, a giant filamentous protein extending along the entire length of the thin filament, provides a blueprint for muscle thin filament assembly. To test this hypothesis, we generated a KO mouse model to investigate nebulin functions in vivo. Nebulin KO mice assemble thin filaments of reduced lengths and approximately 15% of their Z-disks are ab ...[more]

PMID: 16902413

Similar Datasets

The effect of Nebulin-Deficiency on Skeletal Muscle

Project description: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. 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.

2015-06-25 | E-GEOD-70213 | biostudies-arrayexpress

In vivo and in vitro investigations of heterozygous nebulin knock-out mice reveal similarities with mild human form of nemaline myopathy [mRNA]

Project description:In the present study, we used a multimodal approach including protein and gene expression analysis and combined in vivo and in vitro measurements of force production. Overall, we aimed at investigating the functional impact of the expression of a single nebulin allele. Comparison of the mRNA expression profile of quadriceps in 3 nebulin wild type mice with 3 nebulin heterozygous mice.

2012-09-01 | E-GEOD-36743 | biostudies-arrayexpress

In vivo and in vitro investigations of heterozygous nebulin knock-out mice reveal similarities with mild human form of nemaline myopathy [miRNA]

Project description:In the present study, we used a multimodal approach including protein and gene expression analysis and combined in vivo and in vitro measurements of force production. Overall, we aimed at investigating the functional impact of the expression of a single nebulin allele. Comparison of the miRNA expression profile of quadriceps in 3 nebulin wild type mice with 3 nebulin heterozygous mice.

2012-09-01 | E-GEOD-36741 | biostudies-arrayexpress

The effect of Nebulin-Deficiency on Skeletal Muscle

2015-06-25 | GSE70213 | GEO

Transcription profiling of mouse erythroid progenitors (EP) in comparison to granulocyte/monocyte - macrophage progenitors (GMP) from 10 - 16 week old C57/Bl6 - S129Ola

Project description:Mouse erythroid progenitors (EP) in comparison to granulocyte/monocyte - macrophage progenitors (GMP) from 10 - 16 week old C57/Bl6 - S129Ola (mixed genetic background) purified by flow cytometry

2008-06-11 | E-GEOD-1584 | biostudies-arrayexpress

Genetic and epigenetic variation among inbred littermates: identification of metastable epialleles in the mouse

Project description:Genomic DNA was extracted from the livers of 5 22week old male mice, bisulfite converted and sequenced to ~30x coverage. The mice were obtained from two litters from an inbred agouti viable yellow colony. The individuals were isogenic but differed in their coat phenotype with one having a yellow coat, one a psuedoagouti coat and the remaining were phenotypically C57/BL6. The resulting DNA metylation profiles were investigated for individual differences in DNA methylation, including the identificaiton of novel metastable epialleles.

2015-12-01 | GSE72177 | GEO

Transcription profiling of mouse fibroblast cells infected with adenoviral vector or mock infected

Project description:The innate immune response to 1st generation adenoviral vectors was ascertained through high level infection of primary cells from a inbred mouse strain, replete with an intact and functioning TLR system. Experiment Overall Design: Quiescent murine C57/Bl6 fibroblasts were Ad (E1-E3-LacZ at MOI=300) or Mock infected. Sixteen hrs post-infection, RNA was harvested, transcribe to cDNA and bitotin-labeled, and hybridized to Mu11k A and B arrays.

2007-12-04 | E-GEOD-3172 | biostudies-arrayexpress

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

Project description:Genome-wide gene expression analysis on tibialis anterior muscle from 2-month-old nebulin SH3 domain deleted (Neb∆SH3) mice compared to wildtype.

2013-09-23 | GSE47801 | GEO

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

Project description:Genome-wide gene expression analysis on tibialis anterior muscle from 2-month-old nebulin SH3 domain deleted (Neb∆SH3) mice compared to wildtype. Total RNA was obtained from biological triplicates of tibialis anterior muscle from 2-month-old nebulin SH3 domain deleted (Neb∆SH3) mice compared to wildtype.

2013-09-23 | E-GEOD-47801 | biostudies-arrayexpress

Gene expression of transplanted hearts

Project description:C57/BL6 to C57/BL6 Balb/c to C57/BL6 Balb/c to C57/BL6 with anti CD80/86 mAb Identificaton of gene expression profile in tolerizing murine cardiac allograft by co-stimulatory blockade. The induction of specific tolerance would be the ultimate achievement in transplant immunology, but the precise mechanisms of immunological tolerance remain largely unknown. Here, we investigated global gene expression analysis in tolerizing murine cardiac allografts by means of oligonucleotide microarrays. Tolerance induction was achieved in cardiac allografts from BALB/c to C57BL/6 mice by daily intraperitoneal injection of anti-CD80 and CD86 mAbs. Comparative analysis revealed 64 genes to be induced more extensively in the tolerizing than in the syngeneic isografts, and 16 genes than in the rejecting allografts. Two genes were specifically upregulated in the tolerizing allografts. In the tolerizing allografts there were induced marked expressions of a number of genes for proinflammatory factors, including interferon-gamma inducible cytokines and chemokines, as well as apoptosis-related genes which were also upregulated in the rejecting allografts. Moreover, these gene expression patterns continued to be upregulated more than 70 days post transplant. These results provide evidence that immunological tolerance can be induced and maintained in the presence of prominent proinflammatory gene expression in vivo. Keywords: other

2003-10-14 | GSE582 | GEO

OmicsDI is part of the ELIXIR infrastructure

OmicsDI is an Elixir interoperability service. Learn more ›

OmicsDI Databases

PRIDE
PeptideAtlas
MassIVE
JPOST Repository
Physiome Model Repository

EGA
EVA
ENA
LINCS
PAXDB
Cell Collective

MetaboLights
Metabolomics Workbench
MetabolomeExpress
GNPS
BioModels
FAIRDOMHub

ArrayExpress
dbGaP
ExpressionAtlas
GEO
NODE

Information

Databases
Help
API
Contact us
Code on GitHub
Terms of use
Submit Data