PXD007881 is an
original dataset announced via ProteomeXchange.
Dataset Summary
Title | Disease-linked lysine glutarylation impairs function and interactions of mitochondrial proteins and contribute to mitochondrial heterogeneity |
Description | Inherited deficiencies of the lysine and tryptophan catabolic pathways, due to mutations in the glutaryl-CoA-dehydrogenase (GCDH) gene, cause glutaric aciduria type 1 (GA1). In mammals two metabolic routes for L-lysine oxidation exist, the mitochondrial saccharopine pathway, which is predominant in extracerebral tissue and the peroxysomal/cytosolic pipecolate pathway, the main pathway in adult brain. A unique feature of all pathways is the formation of glutaryl-CoA that is converted into crotonyl-CoA via GCDH. In this study we identified increased lysine glutarylation, a novel reversible post-translational modification, in various tissues and cultured cells of Gcdh KO mice as a result of glutaryl-CoA accumulation. Our analysis revealed that glutarylation is widespread in mitochondria. By using a proteomic approach, we identified 37 and 154 glutarylated mitochondrial proteins in brain and liver tissue from Gcdh KO mice, respectively. We further showed that glutarylation supresses glutamate dehydrogenase (GDH) and carbonic anhydrase 5B activity, plus the direct interaction between GCDH and GDH. |
HostingRepository | PRIDE |
AnnounceDate | 2018-07-11 |
AnnouncementXML | Submission_2018-07-11_08:52:35.xml |
DigitalObjectIdentifier | |
ReviewLevel | Peer-reviewed dataset |
DatasetOrigin | Original dataset |
RepositorySupport | Unsupported dataset by repository |
PrimarySubmitter | Marc Sylvester |
SpeciesList | scientific name: Mus musculus (Mouse); NCBI TaxID: 10090; |
ModificationList | No PTMs are included in the dataset |
Instrument | LTQ Orbitrap Velos |
Dataset History
Revision | Datetime | Status | ChangeLog Entry |
0 | 2017-10-02 01:36:41 | ID requested | |
1 | 2018-07-11 03:51:33 | announced | |
⏵ 2 | 2018-07-11 08:52:36 | announced | Updated project metadata. |
3 | 2018-09-17 00:13:31 | announced | Updated project metadata. |
Publication List
Dataset with its publication pending |
Keyword List
curator keyword: Biological |
submitter keyword: glutaryl-CoA-dehydrogenase, glutaryl-proteome, glutamate dehydrogenase, protein interaction, Post-translational Modifications, Proteolysis & Proteomics, Metabolism, Molecular Biology of Disease |
Contact List
Thomas Braulke |
contact affiliation | University Medical Center Hamburg-Eppendorf, ChildrenĀ“s Hospital Department of Biochemistry Hamburg, Germany |
contact email | braulke@uke.de |
lab head | |
Marc Sylvester |
contact affiliation | University of Bonn, Institute of Biochemistry and Molecular Biology |
contact email | msylvest@uni-bonn.de |
dataset submitter | |
Full Dataset Link List
Dataset FTP location
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PRIDE project URI |
Repository Record List
[ + ]
[ - ]
- PRIDE
- PXD007881
- Label: PRIDE project
- Name: Disease-linked lysine glutarylation impairs function and interactions of mitochondrial proteins and contribute to mitochondrial heterogeneity