Summary of Study ST003126

This data is available at the NIH Common Fund's National Metabolomics Data Repository (NMDR) website, the Metabolomics Workbench, https://www.metabolomicsworkbench.org, where it has been assigned Project ID PR001944. The data can be accessed directly via it's Project DOI: 10.21228/M8FD9G This work is supported by NIH grant, U2C- DK119886.

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This study contains a large results data set and is not available in the mwTab file. It is only available for download via FTP as data file(s) here.

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Study IDST003126
Study TitleEffect of high fat diet on heart metabolome of CHCHD10 mutant mice
Study SummaryMutations in CHCHD10, a mitochondrial protein with undefined functions, are associated with autosomal dominant mitochondrial diseases. Chchd10 knock-in mice harboring a heterozygous S55L mutation (equivalent to human pathogenic S59L) develop a fatal mitochondrial cardiomyopathy caused by CHCHD10 aggregation and proteotoxic mitochondrial integrated stress response (mtISR). In mutant hearts, mtISR is accompanied by a metabolic rewiring characterized by increased reliance on glycolysis rather than fatty acid oxidation. To counteract this metabolic rewiring, heterozygous S55L mice were subjected to chronic high fat diet (HFD) to decrease insulin sensitivity and glucose uptake and enhance fatty acid utilization in the heart. HFD ameliorated the ventricular dysfunction of mutant hearts and significantly extended the survival of mutant female mice affected by severe pregnancy-induced cardiomyopathy. Gene expression profiles confirmed that HFD increased fatty acid utilization and ameliorated cardiomyopathy markers. Importantly, HFD also decreased accumulation of aggregated CHCHD10 in the S55L heart, suggesting activation of quality control mechanisms. Overall, our findings indicate that metabolic therapy can be effective in mitochondrial cardiomyopathies associated with proteotoxic stress.
Institute
Weill Cornell Medicine
Last NameSouthwell
First NameNneka
Address407 E 61st St
Emailnns4001@med.cornell.edu
Phone646-962-8172
Submit Date2024-03-03
Raw Data AvailableYes
Raw Data File Type(s)raw(Thermo)
Analysis Type DetailLC-MS
Release Date2024-03-26
Release Version1
Nneka Southwell Nneka Southwell
https://dx.doi.org/10.21228/M8FD9G
ftp://www.metabolomicsworkbench.org/Studies/ application/zip

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Factors:

Subject type: Mammal; Subject species: Mus musculus (Factor headings shown in green)

mb_sample_id local_sample_id Sample source Genotype Sex Diet
SA338887H_HetCD_1Heart CHCHD10 S55L Het F Control Diet
SA338888H_HetCD_4Heart CHCHD10 S55L Het F Control Diet
SA338889H_HetCD_5Heart CHCHD10 S55L Het F Control Diet
SA338890H_HetCD_3Heart CHCHD10 S55L Het F Control Diet
SA338891H_HetCD_2Heart CHCHD10 S55L Het F Control Diet
SA338892H_HetHFD_2Heart CHCHD10 S55L Het F High Fat Diet
SA338893H_HetHFD_3Heart CHCHD10 S55L Het F High Fat Diet
SA338894H_HetHFD_5Heart CHCHD10 S55L Het F High Fat Diet
SA338895H_HetHFD_4Heart CHCHD10 S55L Het F High Fat Diet
SA338896H_HetHFD_1Heart CHCHD10 S55L Het F High Fat Diet
SA338897H_HetCD_10Heart CHCHD10 S55L Het M Control Diet
SA338898H_HetCD_8Heart CHCHD10 S55L Het M Control Diet
SA338899H_HetCD_9Heart CHCHD10 S55L Het M Control Diet
SA338900H_HetCD_6Heart CHCHD10 S55L Het M Control Diet
SA338901H_HetCD_7Heart CHCHD10 S55L Het M Control Diet
SA338902H_HetHFD_8Heart CHCHD10 S55L Het M High Fat Diet
SA338903H_HetHFD_10Heart CHCHD10 S55L Het M High Fat Diet
SA338904H_HetHFD_9Heart CHCHD10 S55L Het M High Fat Diet
SA338905H_HetHFD_7Heart CHCHD10 S55L Het M High Fat Diet
SA338906H_HetHFD_6Heart CHCHD10 S55L Het M High Fat Diet
SA338907H_WTCD_5Heart WT F Control Diet
SA338908H_WTCD_4Heart WT F Control Diet
SA338909H_WTCD_1Heart WT F Control Diet
SA338910H_WTCD_2Heart WT F Control Diet
SA338911H_WTCD_3Heart WT F Control Diet
SA338912H_WTHFD_5Heart WT F High Fat Diet
SA338913H_WTHFD_2Heart WT F High Fat Diet
SA338914H_WTHFD_3Heart WT F High Fat Diet
SA338915H_WTHFD_4Heart WT F High Fat Diet
SA338916H_WTHFD_1Heart WT F High Fat Diet
SA338917H_WTCD_10Heart WT M Control Diet
SA338918H_WTCD_6Heart WT M Control Diet
SA338919H_WTCD_9Heart WT M Control Diet
SA338920H_WTCD_7Heart WT M Control Diet
SA338921H_WTCD_8Heart WT M Control Diet
SA338922H_WTHFD_10Heart WT M High Fat Diet
SA338923H_WTHFD_9Heart WT M High Fat Diet
SA338924H_WTHFD_6Heart WT M High Fat Diet
SA338925H_WTHFD_7Heart WT M High Fat Diet
SA338926H_WTHFD_8Heart WT M High Fat Diet
Showing results 1 to 40 of 40
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