PXD050914 is an
original dataset announced via ProteomeXchange.
Dataset Summary
Title | T he proteostasis interactomes of trafficking - deficient K V 11.1 variants associated with Long QT Syndrome and pharmacological chaperone rescue |
Description | The voltage gated potassium ion channel KV11.1 plays a critical role in cardiac repolarization. Genetic variants that render Kv11.1 dysfunctional cause Long QT Syndrome (LQTS), which is associated with fatal arrhythmias. Approximately 90% of LQTS-associated variants cause intracellular protein transport (trafficking) dysfunction, which can be rescued by pharmacological chaperones like E-4031. Protein folding and trafficking decisions are regulated by chaperones, protein quality control factors, and trafficking machinery comprising the cellular proteostasis network. Here, we test whether trafficking dysfunction is associated with alterations in the proteostasis network of pathogenic Kv11.1 variants, and whether pharmacological chaperones can normalize the proteostasis network of responsive variants. |
HostingRepository | PRIDE |
AnnounceDate | 2024-10-22 |
AnnouncementXML | Submission_2024-10-22_06:54:37.966.xml |
DigitalObjectIdentifier | |
ReviewLevel | Peer-reviewed dataset |
DatasetOrigin | Original dataset |
RepositorySupport | Unsupported dataset by repository |
PrimarySubmitter | Christian Egly |
SpeciesList | scientific name: Homo sapiens (Human); NCBI TaxID: 9606; |
ModificationList | iodoacetamide derivatized residue |
Instrument | Orbitrap Exploris 480 |
Dataset History
Revision | Datetime | Status | ChangeLog Entry |
0 | 2024-03-22 16:05:16 | ID requested | |
1 | 2024-08-10 16:25:06 | announced | |
⏵ 2 | 2024-10-22 06:54:38 | announced | 2024-10-22: Updated project metadata. |
Publication List
Egly CL, Barny LA, Do T, McDonald EF, Knollmann BC, Plate L, 11.1 associated with long QT syndrome. J Biol Chem, 300(7):107465(2024) [pubmed] |
10.1016/j.jbc.2024.107465; |
Keyword List
submitter keyword: hERG, Long QT Syndrome,ion channel, trafficking, K V 11.1, interactome |
Contact List
Lars Plate |
contact affiliation | Vanderbilt University |
contact email | lars.plate@vanderbilt.edu |
lab head | |
Christian Egly |
contact affiliation | Vanderbilt University Medical Center |
contact email | christian.l.egly@vumc.org |
dataset submitter | |
Full Dataset Link List
Dataset FTP location
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PRIDE project URI |
Repository Record List
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[ - ]
- PRIDE
- PXD050914
- Label: PRIDE project
- Name: T he proteostasis interactomes of trafficking - deficient K V 11.1 variants associated with Long QT Syndrome and pharmacological chaperone rescue