Summary of Study ST004190

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 PR002642. The data can be accessed directly via it's Project DOI: 10.21228/M84V8S This work is supported by NIH grant, U2C- DK119886. See: https://www.metabolomicsworkbench.org/about/howtocite.php

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Study IDST004190
Study TitleComparative Analysis of the Metabolic Profiles of Alix−/− and Ozz−/− Soleus Skeletal Muscle
Study TypeMetabolomics
Study SummarySkeletal muscle relies on mitochondrial proteostasis for energy production, which is maintained by two key quality-control systems: the ubiquitin-proteasome system (UPS) and mitochondria-derived vesicles. This study reveals that the E3 ligase complex CRL5Ozz and its substrate Alix both localize to mitochondria and cooperate to regulate the mitochondrial ATP transporter Slc25A4. CRL5Ozz targets Slc25A4 for proteasomal degradation, while Alix promotes Slc25A4 loading into exosomes for lysosomal degradation. Loss of Ozz or Alix in mice disrupts Slc25A4 levels, causing mitochondrial dysfunction, altered metabolism, and a shift in muscle fiber type from oxidative to glycolytic. This work uncovers a coordinated role of UPS and vesicle-mediated degradation in mitochondrial quality control.
Institute
St Jude Children's Research Hospital
DepartmentGenetics
LaboratoryAlessandra d'Azzo Lab
Last NamePalacios
First NameGustavo
Address262 Danny Thomas Place, Memphis, TN 38105.
EmailGustavo.Palacios@stjude.org
Phone901-595-4448
Submit Date2025-09-08
Num Groups3
Total Subjects14
Num Males6
Num Females8
Study CommentsWe did not observe any differences in the results due to the animal's gender
Raw Data AvailableYes
Raw Data File Type(s)mzML
Analysis Type DetailLC-MS
Release Date2025-10-06
Release Version1
Gustavo Palacios Gustavo Palacios
https://dx.doi.org/10.21228/M84V8S
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 Genotype Sample source
SA483383Alix_R4_NegAlix-KO Soleus
SA483384Alix_R1_PosAlix-KO Soleus
SA483385Alix_R4_PosAlix-KO Soleus
SA483386Alix_R1_NegAlix-KO Soleus
SA483387Alix_R3_PosAlix-KO Soleus
SA483388Alix_R2_NegAlix-KO Soleus
SA483389Alix_R2_PosAlix-KO Soleus
SA483390Alix_R3_NegAlix-KO Soleus
SA483391Ozz_R6_PosOzz-KO Soleus
SA483392Ozz_R6_NegOzz-KO Soleus
SA483393Ozz_R5_NegOzz-KO Soleus
SA483394Ozz_R4_PosOzz-KO Soleus
SA483395Ozz_R5_PosOzz-KO Soleus
SA483396Ozz_R3_PosOzz-KO Soleus
SA483397Ozz_R3_NegOzz-KO Soleus
SA483398Ozz_R2_PosOzz-KO Soleus
SA483399Ozz_R2_NegOzz-KO Soleus
SA483400Ozz_R1_PosOzz-KO Soleus
SA483401Ozz_R1_NegOzz-KO Soleus
SA483402Ozz_R4_NegOzz-KO Soleus
SA483403WT_R1_NegWT Soleus
SA483404WT_R1_PosWT Soleus
SA483405WT_R2_NegWT Soleus
SA483406WT_R2_PosWT Soleus
SA483407WT_R3_NegWT Soleus
SA483408WT_R3_PosWT Soleus
SA483409WT_R4_NegWT Soleus
SA483410WT_R4_PosWT Soleus
Showing results 1 to 28 of 28
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