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GEO help: Mouse over screen elements for information. |
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| Status |
Public on Jul 31, 2024 |
| Title |
Expression data from adult Dele1 and OMA1 mouse brown adipose tissues (BATs). |
| Organism |
Mus musculus |
| Experiment type |
Expression profiling by array
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| Summary |
Mitochondrial dysfunction causes devastating disorders, including mitochondrial myopathy. Here, we identified that diverse mitochondrial myopathy models elicit a protective mitochondrial integrated stress response (mt-ISR), mediated by OMA1-DELE1 signaling. The response was similar following disruptions in mtDNA maintenance, from knockout of Tfam, and mitochondrial protein unfolding, from disease-causing mutations in CHCHD10 (G58R and S59L). The preponderance of the response was directed at upregulating pathways for aminoacyl-tRNA biosynthesis, the intermediates for protein synthesis, and was similar in heart and skeletal muscle but more limited in brown adipose challenged with cold stress. Strikingly, models with early DELE1 mt-ISR activation failed to grow and survive to adulthood in the absence of Dele1, accounting for some but not all of OMA1’s protection. Notably, the DELE1 mt-ISR did not slow net protein synthesis in stressed striated muscle, but instead prevented loss of translation-associated proteostasis in muscle fibers. Together our findings identify that the DELE1 mt-ISR mediates a stereotyped response to diverse forms of mitochondrial stress and is particularly critical for maintaining growth and survival in early-onset mitochondrial myopathy.
This experiment used the Clariom_S_Mouse Microarray from Affymetrix/Applied Biosystems to analyze the effect of Dele1 KO and OMA1 KO under cold stress.
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| Overall design |
42 total samples were analyzed (7 biological replicates of each of the 3 different genotypes each in 2 different condition). Genes with an FDR≤0.05 and a fold-change ≥2 were selected.
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| Web link |
https://doi.org/10.1038/s44318-024-00242-x
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| Contributor(s) |
Narendra D, Lin H |
| Citation(s) |
39379554 |
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| Submission date |
Jul 26, 2024 |
| Last update date |
Oct 30, 2024 |
| Contact name |
Narendra Derek |
| E-mail(s) |
derek.narendra@nih.gov
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| Phone |
301-594-4737
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| Organization name |
National Institutes of Health
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| Department |
National Institute of Neurological Disorders and Stroke
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| Lab |
Inherited Movement Disorders Unit, Neurogenetics Branch
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| Street address |
35 Convent Drive, Room 2A215
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| City |
Bethesda |
| State/province |
MD |
| ZIP/Postal code |
20814 |
| Country |
USA |
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| Platforms (1) |
| GPL23038 |
[Clariom_S_Mouse] Affymetrix Clariom S Assay, Mouse (Includes Pico Assay) |
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| Samples (42)
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| GSM8423922 |
Adult OMA1 KO BAT, cold, biological rep1 |
| GSM8423923 |
Adult OMA1 KO BAT, cold, biological rep2 |
| GSM8423924 |
Adult OMA1 KO BAT, RT, biological rep1 |
| GSM8423925 |
Adult OMA1 KO BAT, cold, biological rep3 |
| GSM8423926 |
Adult OMA1 KO BAT, cold, biological rep4 |
| GSM8423927 |
Adult OMA1 KO BAT, cold, biological rep5 |
| GSM8423928 |
Adult OMA1 KO BAT, RT, biological rep2 |
| GSM8423929 |
Adult OMA1 KO BAT, RT, biological rep3 |
| GSM8423930 |
Adult OMA1 KO BAT, RT, biological rep4 |
| GSM8423931 |
Adult OMA1 KO BAT, cold, biological rep6 |
| GSM8423932 |
Adult OMA1 KO BAT, RT, biological rep5 |
| GSM8423933 |
Adult OMA1 KO BAT, RT, biological rep6 |
| GSM8423934 |
Adult OMA1 KO BAT, RT, biological rep7 |
| GSM8423935 |
Adult OMA1 KO BAT, cold, biological rep7 |
| GSM8423936 |
Adult WT BAT, RT, biological rep1 |
| GSM8423937 |
Adult Dele1 KO BAT, RT, biological rep1 |
| GSM8423938 |
Adult Dele1 KO BAT, RT, biological rep2 |
| GSM8423939 |
Adult WT BAT, RT, biological rep2 |
| GSM8423940 |
Adult WT BAT, RT, biological rep3 |
| GSM8423941 |
Adult WT BAT, RT, biological rep4 |
| GSM8423942 |
Adult WT BAT, RT, biological rep5 |
| GSM8423943 |
Adult Dele1 KO BAT, RT, biological rep3 |
| GSM8423944 |
Adult WT BAT, RT, biological rep6 |
| GSM8423945 |
Adult Dele1 KO BAT, RT, biological rep4 |
| GSM8423946 |
Adult WT BAT, RT, biological rep7 |
| GSM8423947 |
Adult Dele1 KO BAT, RT, biological rep5 |
| GSM8423948 |
Adult Dele1 KO BAT, RT, biological rep6 |
| GSM8423949 |
Adult Dele1 KO BAT, RT, biological rep7 |
| GSM8423950 |
Adult Dele1 KO BAT, cold, biological rep1 |
| GSM8423951 |
Adult WT BAT, cold, biological rep1 |
| GSM8423952 |
Adult WT BAT, cold, biological rep2 |
| GSM8423953 |
Adult Dele1 KO BAT, cold, biological rep2 |
| GSM8423954 |
Adult Dele1 KO BAT, cold, biological rep3 |
| GSM8423955 |
Adult WT BAT, cold, biological rep3 |
| GSM8423956 |
Adult WT BAT, cold, biological rep4 |
| GSM8423957 |
Adult WT BAT, cold, biological rep5 |
| GSM8423958 |
Adult Dele1 KO BAT, cold, biological rep4 |
| GSM8423959 |
Adult WT BAT, cold, biological rep6 |
| GSM8423960 |
Adult Dele1 KO BAT, cold, biological rep5 |
| GSM8423961 |
Adult WT BAT, cold, biological rep7 |
| GSM8423962 |
Adult Dele1 KO BAT, cold, biological rep6 |
| GSM8423963 |
Adult Dele1 KO BAT, cold, biological rep7 |
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| Relations |
| BioProject |
PRJNA1140595 |
| Supplementary file |
Size |
Download |
File type/resource |
| GSE273199_RAW.tar |
61.0 Mb |
(http)(custom) |
TAR (of CEL, CHP) |
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