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| Status |
Public on Feb 08, 2024 |
| Title |
fhl2b expression ameliorates muscular dystrophy [5 months EOM and trunk] |
| Organism |
Danio rerio |
| Experiment type |
Expression profiling by high throughput sequencing
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| Summary |
In muscle dystrophies, muscle fibers loose integrity and die, leading to significant suffering and a shorter life. Strikingly, the extraocular muscles (EOMs), controlling eye movements, are spared and function well despite the disease progression. Although EOMs have been shown to have important differences compared to body musculature the mechanisms underlying this inherent resistance to muscle dystrophies remain largely unknown. Here, we demonstrate important differences in gene expression as a response to muscle dystrophies between the EOMs and trunk muscle in zebrafish via transcriptomic profiling. We show that the LIM-protein Fhl2 is upregulated in response to knockout of desmin, plectin and obscurin, intermediate filament proteins causing different muscle dystrophies, and contributes to disease protection of the EOMs. Moreover, we show that ectopic expression of fhl2b can partially rescue the muscle phenotype in the zebrafish Duchenne muscular dystrophy model sapje, significantly improving their survival rate. Therefore, fhl2 is a protective agent and a candidate target gene for therapy of muscle dystrophies.
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| Overall design |
To examine the innate resistance of extraocular muscle to muscular dystrophies we knocked down desmin and sequenced extraocular muscle as well as trunk muscle from zebrafish at two time points. We then performed gene expression analysis between WT and desmin knock-down tissues of the same type at the same time points.
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| Contributor(s) |
Dennhag N, Kahsay A, Nissen I, Cherminina M, Nord H, Liu J, Arner A, Backman L, Remeseiro S, von Hofsten J, Pedrosa Domellöf F |
| Citation(s) |
38431640 |
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| Submission date |
Sep 01, 2023 |
| Last update date |
Mar 13, 2024 |
| Contact name |
Nils Dennhag |
| E-mail(s) |
nils.dennhag@umu.se
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| Organization name |
Umeå Univeristy
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| Street address |
Linneaus väg 9
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| City |
Umeå |
| ZIP/Postal code |
901 87 |
| Country |
Sweden |
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| Platforms (1) |
| GPL24995 |
Illumina NovaSeq 6000 (Danio rerio) |
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| Samples (12)
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| This SubSeries is part of SuperSeries: |
| GSE242137 |
fhl2b expression ameliorates muscular dystrophy |
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| Relations |
| BioProject |
PRJNA1011841 |
| Supplementary file |
Size |
Download |
File type/resource |
| GSE242136_RAW.tar |
2.8 Mb |
(http)(custom) |
TAR (of TAB) |
SRA Run Selector |
| Raw data are available in SRA |
| Processed data provided as supplementary file |
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