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PXD028286 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleReprograming of Proteasomal Degradation by Branched Chain Amino Acid Metabolism
DescriptionBranched chain amino acids (BCAAs) play an important role in energy and protein regulation. Defects in BCAA metabolism are linked to numerous pathologies, including diabetes, neurodegeneration, and premature aging. Isovaleric acidemia is a metabolic disease caused by defective leucine breakdown and an abnormal accumulation of isovaleric acid in the body fluids; however, the cytotoxic effects caused by excess isovaleric acid remained unclear. Here, we provide a regulatory connection between BCAA metabolism and the ubiquitin/proteasome-system (UPS) in Caenorhabditis elegans. Multi-omic analysis identified that worms lacking the isovaleryl-CoA dehydrogenase IVD-1 exhibit reduced expression of regulatory proteasome subunits and defects in ubiquitin-dependent proteolysis. Conversely, proteasomal protein degradation was supported by the branched chain amino transferase BCAT-1. Adding extra isovaleric acid to the growth medium triggered UPS defects, implying a causative role of perturbed proteostasis in isovaleric academia.
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterPrerana Wagle
SpeciesList scientific name: Caenorhabditis elegans; NCBI TaxID: 6239;
ModificationListiodoacetamide derivatized residue
InstrumentQ Exactive
Dataset History
RevisionDatetimeStatusChangeLog Entry
02021-09-09 08:10:49ID requested
12022-09-22 09:38:52announced
Publication List
Dataset with its publication pending
Keyword List
submitter keyword: C. elegans
leucine catabolism
branched chain amino acid (BCAA)
Isovaleric Acidemia
Contact List
Thorsten Hoppe
contact affiliationCECAD Research Center, University of Cologne ,Institute of Genetics, Cologne, Germany
contact emailthorsten.hoppe@uni-koeln.de
lab head
Prerana Wagle
contact affiliationCECAD Research Center
contact emailproteomics-facility@uni-koeln.de
dataset submitter
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Dataset FTP location
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