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PXD006023

PXD006023 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleAGK human LC-MSMS project part I (proteome)
DescriptionMutations in the mitochondrial acylglycerol kinase AGK cause Sengers syndrome characterized by cataracts, hypertrophic cardiomyopathy and skeletal myopathy. AGK generates phosphatidic acid and lyso-phosphatidic acid, bioactive phospholipids involved in lipid signaling and the regulation of tumor progression. However, molecular mechanisms of the mitochondrial pathology remain enigmatic. Determining its mitochondrial interactome, we have identified AGK as a constituent of the TIM22 protein translocase in the mitochondrial inner membrane. AGK assembles with TIMM22 and TIMM29 and supports the import of a subset of multi-spanning membrane proteins. The function of AGK as subunit of the TIM22 translocase does not depend on its kinase activity. However, enzymatically active AGK is required to maintain mitochondrial cristae morphogenesis and the apoptotic resistance of cells. The dual function of AGK as lipid kinase and constituent of the TIM22 translocase reveals that disturbances in both phospholipid metabolism and mitochondrial protein biogenesis contribute to the pathogenesis of Sengers syndrome. See the published paper for more information of the experimental setup.
HostingRepositoryPRIDE
AnnounceDate2024-10-22
AnnouncementXMLSubmission_2024-10-22_04:07:17.541.xml
DigitalObjectIdentifier
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterHendrik Nolte
SpeciesList scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationListNo PTMs are included in the dataset
InstrumentQ Exactive
Dataset History
RevisionDatetimeStatusChangeLog Entry
02017-03-06 01:17:53ID requested
12017-08-14 04:11:25announced
22024-10-22 04:07:19announced2024-10-22: Updated project metadata.
Publication List
10.1016/j.molcel.2017.06.013;
Vukotic M, Nolte H, K, ö, nig T, Saita S, Ananjew M, Kr, ü, ger M, Tatsuta T, Langer T, Acylglycerol Kinase Mutated in Sengers Syndrome Is a Subunit of the TIM22 Protein Translocase in Mitochondria. Mol Cell, 67(3):471-483.e7(2017) [pubmed]
Keyword List
curator keyword: Biomedical
submitter keyword: AGK, human, LC-MS/MS
Contact List
Marcus Krüger
contact affiliationInstitute for Genetics and Cologne Excellence Cluster on Cellular Stress Responses in Aging-Associated Diseases (CECAD), University of Cologne, Cologne, Germany
contact emailmarcus.krueger@uni-koeln.de
lab head
Hendrik Nolte
contact affiliationInstitute for Genetics and Cologne Excellence Cluster on Cellular Stress Responses in Aging-Associated Diseases (CECAD)
contact emailh.nolte@uni-koeln.de
dataset submitter
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Dataset FTP location
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PRIDE project URI
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