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
| Study ID | ST004190 |
| Study Title | Comparative Analysis of the Metabolic Profiles of Alix−/− and Ozz−/− Soleus Skeletal Muscle |
| Study Type | Metabolomics |
| Study Summary | Skeletal 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 |
| Department | Genetics |
| Laboratory | Alessandra d'Azzo Lab |
| Last Name | Palacios |
| First Name | Gustavo |
| Address | 262 Danny Thomas Place, Memphis, TN 38105. |
| Gustavo.Palacios@stjude.org | |
| Phone | 901-595-4448 |
| Submit Date | 2025-09-08 |
| Num Groups | 3 |
| Total Subjects | 14 |
| Num Males | 6 |
| Num Females | 8 |
| Study Comments | We did not observe any differences in the results due to the animal's gender |
| Raw Data Available | Yes |
| Raw Data File Type(s) | mzML |
| Analysis Type Detail | LC-MS |
| Release Date | 2025-10-06 |
| Release Version | 1 |
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Combined analysis:
| Analysis ID | AN006961 | AN006962 |
|---|---|---|
| Chromatography ID | CH005286 | CH005286 |
| MS ID | MS006658 | MS006659 |
| Analysis type | MS | MS |
| Chromatography type | HILIC | HILIC |
| Chromatography system | Thermo Vanquish | Thermo Vanquish |
| Column | Waters ACQUITY UPLC BEH Amide (150 x 1 mm, 1.7 um) | Waters ACQUITY UPLC BEH Amide (150 x 1 mm, 1.7 um) |
| MS Type | ESI | ESI |
| MS instrument type | Orbitrap | Orbitrap |
| MS instrument name | Thermo Q Exactive Orbitrap | Thermo Q Exactive Orbitrap |
| Ion Mode | NEGATIVE | POSITIVE |
| Units | Peak area | Peak area |
