Summary of Study ST003128

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.

See: https://www.metabolomicsworkbench.org/about/howtocite.php

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.

Perform statistical analysis  |  Show all samples  |  Show named metabolites  |  Download named metabolite data  
Download mwTab file (text)   |  Download mwTab file(JSON)   |  Download data files (Contains raw data)
Study IDST003128
Study TitleEffect of high fat diet on serum fatty acids, lipidome and metabolome in 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 Date2024-03-06
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

Select appropriate tab below to view additional metadata details:

Factors:

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

mb_sample_id local_sample_id Sample source Genotype Treatment Age (d) Sex
SA338967SL_HetCD_6Serum CHCHD10 S55L Het Control Diet 250 F
SA338968S_HetCD_2Serum CHCHD10 S55L Het Control Diet 250 F
SA338969SL_HetCD_3Serum CHCHD10 S55L Het Control Diet 250 F
SA338970SP_HetCD_1Serum CHCHD10 S55L Het Control Diet 250 F
SA338971SL_HetCD_5Serum CHCHD10 S55L Het Control Diet 250 F
SA338972SL_HetCD_2Serum CHCHD10 S55L Het Control Diet 250 F
SA338973SL_HetCD_1Serum CHCHD10 S55L Het Control Diet 250 F
SA338974SL_HetCD_4Serum CHCHD10 S55L Het Control Diet 250 F
SA338975SP_HetCD_6Serum CHCHD10 S55L Het Control Diet 250 F
SA338976S_HetCD_1Serum CHCHD10 S55L Het Control Diet 250 F
SA338977S_HetCD_5Serum CHCHD10 S55L Het Control Diet 250 F
SA338978S_HetCD_6Serum CHCHD10 S55L Het Control Diet 250 F
SA338979SP_HetCD_2Serum CHCHD10 S55L Het Control Diet 250 F
SA338980S_HetCD_4Serum CHCHD10 S55L Het Control Diet 250 F
SA338981S_HetCD_3Serum CHCHD10 S55L Het Control Diet 250 F
SA338982SP_HetCD_5Serum CHCHD10 S55L Het Control Diet 250 F
SA338983SP_HetCD_4Serum CHCHD10 S55L Het Control Diet 250 F
SA338984SP_HetCD_3Serum CHCHD10 S55L Het Control Diet 250 F
SA338985SL_HetHFD_2Serum CHCHD10 S55L Het High Fat Diet 250 F
SA338986SL_HetHFD_5Serum CHCHD10 S55L Het High Fat Diet 250 F
SA338987SL_HetHFD_6Serum CHCHD10 S55L Het High Fat Diet 250 F
SA338988SL_HetHFD_4Serum CHCHD10 S55L Het High Fat Diet 250 F
SA338989SL_HetHFD_3Serum CHCHD10 S55L Het High Fat Diet 250 F
SA338990SP_HetHFD_1Serum CHCHD10 S55L Het High Fat Diet 250 F
SA338991SL_HetHFD_1Serum CHCHD10 S55L Het High Fat Diet 250 F
SA338992SP_HetHFD_4Serum CHCHD10 S55L Het High Fat Diet 250 F
SA338993S_HetHFD_3Serum CHCHD10 S55L Het High Fat Diet 250 F
SA338994SP_HetHFD_2Serum CHCHD10 S55L Het High Fat Diet 250 F
SA338995S_HetHFD_4Serum CHCHD10 S55L Het High Fat Diet 250 F
SA338996S_HetHFD_5Serum CHCHD10 S55L Het High Fat Diet 250 F
SA338997S_HetHFD_2Serum CHCHD10 S55L Het High Fat Diet 250 F
SA338998S_HetHFD_1Serum CHCHD10 S55L Het High Fat Diet 250 F
SA338999SP_HetHFD_3Serum CHCHD10 S55L Het High Fat Diet 250 F
SA339000SP_HetHFD_5Serum CHCHD10 S55L Het High Fat Diet 250 F
SA339001SP_HetHFD_6Serum CHCHD10 S55L Het High Fat Diet 250 F
SA339002S_HetHFD_6Serum CHCHD10 S55L Het High Fat Diet 250 F
SA339003SP_WTCD_4Serum WT Control Diet 250 F
SA339004SP_WTCD_1Serum WT Control Diet 250 F
SA339005SP_WTCD_2Serum WT Control Diet 250 F
SA339006SP_WTCD_3Serum WT Control Diet 250 F
SA339007SP_WTCD_5Serum WT Control Diet 250 F
SA339008SP_WTCD_6Serum WT Control Diet 250 F
SA339009SL_WTCD_1Serum WT Control Diet 250 F
SA339010SL_WTCD_2Serum WT Control Diet 250 F
SA339011SL_WTCD_3Serum WT Control Diet 250 F
SA339012S_WTCD_2Serum WT Control Diet 250 F
SA339013S_WTCD_3Serum WT Control Diet 250 F
SA339014S_WTCD_5Serum WT Control Diet 250 F
SA339015S_WTCD_4Serum WT Control Diet 250 F
SA339016S_WTCD_6Serum WT Control Diet 250 F
SA339017SL_WTCD_4Serum WT Control Diet 250 F
SA339018SL_WTCD_5Serum WT Control Diet 250 F
SA339019S_WTCD_1Serum WT Control Diet 250 F
SA339020SL_WTCD_6Serum WT Control Diet 250 F
SA339021SP_WTHFD_6Serum WT High Fat Diet 250 F
SA339022SP_WTHFD_5Serum WT High Fat Diet 250 F
SA339023SP_WTHFD_1Serum WT High Fat Diet 250 F
SA339024SP_WTHFD_3Serum WT High Fat Diet 250 F
SA339025SP_WTHFD_2Serum WT High Fat Diet 250 F
SA339026SP_WTHFD_4Serum WT High Fat Diet 250 F
SA339027S_WTHFD_3Serum WT High Fat Diet 250 F
SA339028SL_WTHFD_2Serum WT High Fat Diet 250 F
SA339029SL_WTHFD_6Serum WT High Fat Diet 250 F
SA339030SL_WTHFD_5Serum WT High Fat Diet 250 F
SA339031SL_WTHFD_4Serum WT High Fat Diet 250 F
SA339032SL_WTHFD_1Serum WT High Fat Diet 250 F
SA339033S_WTHFD_6Serum WT High Fat Diet 250 F
SA339034S_WTHFD_2Serum WT High Fat Diet 250 F
SA339035SL_WTHFD_3Serum WT High Fat Diet 250 F
SA339036S_WTHFD_4Serum WT High Fat Diet 250 F
SA339037S_WTHFD_5Serum WT High Fat Diet 250 F
SA339038S_WTHFD_1Serum WT High Fat Diet 250 F
Showing results 1 to 72 of 72
  logo