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PXD018451

PXD018451 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitlePolyQ-expansion does not alter the huntingtin-HAP40 complex
DescriptionThe amplification of a CAG repeat in the gene coding for huntingtin (HTT) leads to Huntington’s disease (HD). At the protein level, this translates into the expansion of a poly-glutamine (polyQ) stretch in the HTT N-terminus, which renders HTT aggregation-prone by unknown mechanisms. Here we investigate the effects of polyQ expansion on the HTT-HAP40 complex, where HTT structure is substantially stabilized. Surprisingly, our biophysical, cryo-EM and crosslinking mass spectrometry experiments reveal no major changes between 17QHTT-HAP40 (wild type), 46QHTT-HAP40 (typical polyQ length in HD patients) and 128QHTT-HAP40 (extreme polyQ length). Thus, the proposed destabilizing effect of HTT polyQ expansion may not suffice to alter the HTT-HAP40 complex, suggesting that polyQ expansion does not have a major global effect on HTT structure.
HostingRepositoryPRIDE
AnnounceDate2021-03-19
AnnouncementXMLSubmission_2021-03-19_06:22:58.430.xml
DigitalObjectIdentifier
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterFlorian Stengel
SpeciesList scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationListmonohydroxylated residue; iodoacetamide derivatized residue
InstrumentOrbitrap Fusion
Dataset History
RevisionDatetimeStatusChangeLog Entry
02020-04-09 07:28:06ID requested
12021-03-19 06:22:58announced
Publication List
Dataset with its publication pending
Keyword List
submitter keyword: Huntingtin
Huntington’s disease
polyglutamine expansion
cryo-electron microscopy
crosslinking mass spectrometry
Contact List
Florian Stengel
contact affiliationDepartment of Biology, Cellular Proteostasis and Mass Sepectrometry, University of Konstanz
contact emailflorian.stengel@uni-konstanz.de
lab head
Florian Stengel
contact affiliationUniversität Konstanz
contact emailflorian.stengel@uni-konstanz.de
dataset submitter
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Dataset FTP location
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