PXD028286-2

PXD028286 is an original dataset announced via ProteomeXchange.

Title	Reprograming of Proteasomal Degradation by Branched Chain Amino Acid Metabolism
Description	Branched 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.
HostingRepository	PRIDE
AnnounceDate	2024-10-22
AnnouncementXML	Submission_2024-10-22_05:39:45.635.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Prerana Wagle
SpeciesList	scientific name: Caenorhabditis elegans; NCBI TaxID: 6239;
ModificationList	iodoacetamide derivatized residue
Instrument	Q Exactive

Revision	Datetime	Status	ChangeLog Entry
0	2021-09-09 08:10:49	ID requested
1	2022-09-22 09:38:52	announced
⏵ 2	2024-10-22 05:39:46	announced	2024-10-22: Updated project metadata.

Dataset with its publication pending

submitter keyword: C. elegans

ubiquitin

leucine catabolism

branched chain amino acid (BCAA)

BCAT

IVD

Isovaleric Acidemia

Thorsten Hoppe
contact affiliation	CECAD Research Center, University of Cologne ,Institute of Genetics, Cologne, Germany
contact email	thorsten.hoppe@uni-koeln.de
lab head
Prerana Wagle
contact affiliation	CECAD Research Center
contact email	proteomics-facility@uni-koeln.de
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD028286
     1. Label: PRIDE project
     2. Name: Reprograming of Proteasomal Degradation by Branched Chain Amino Acid Metabolism