PXD006849 is an
original dataset announced via ProteomeXchange.
Dataset Summary
Title | ZMYND10 functions in a chaperone-relay during axonemal dynein assembly |
Description | Molecular chaperones are abundant cellular proteins that promote the folding, function and macromolecular assembly of a diverse set of substrate proteins, also known as clients. Regulation of chaperone specificity and function involves association with a growing set of distinct cofactors, or cochaperones. How these client, co-chaperone and chaperone interactions in vivo underpin the proteostasis network during development and disease remains unclear. We have combined mouse genetics, imaging and quantitative proteomics to systematically characterize a chaperone-cochaperone-client interaction network in mammalian motile ciliated cells. We uncover ZMYND10 to act as a specificity factor for the immunophilin cochaperone FKBP8 and HSP90 chaperone for the biosynthesis of the heavy chains of axonemal dyneins required for cilial beat. We establish a series of protein-protein interactions during cytoplasmic pre-assembly of macromolecular dynein motors, some of which are directly ZMYND10-dependent. In the absence of ZMYND10, failure of these interactions results in misfolded and unstable dynein heavy chains, which together with non-functional folding intermediates, are targeted for degradation. We propose that the motile ciliopathy Primary Ciliary Dyskinesia (PCD), such as seen in Zmynd10 mutants, be reconsidered as a disorder of protein folding, which opens new avenues for development of therapeutics. |
HostingRepository | PRIDE |
AnnounceDate | 2024-10-22 |
AnnouncementXML | Submission_2024-10-22_04:38:51.268.xml |
DigitalObjectIdentifier | |
ReviewLevel | Peer-reviewed dataset |
DatasetOrigin | Original dataset |
RepositorySupport | Unsupported dataset by repository |
PrimarySubmitter | Alex von kriegsheim |
SpeciesList | scientific name: Mus musculus (Mouse); NCBI TaxID: 10090; |
ModificationList | No PTMs are included in the dataset |
Instrument | Q Exactive |
Dataset History
Revision | Datetime | Status | ChangeLog Entry |
0 | 2017-06-30 05:27:32 | ID requested | |
1 | 2018-07-12 06:36:54 | announced | |
⏵ 2 | 2024-10-22 04:38:51 | announced | 2024-10-22: Updated project metadata. |
Publication List
Dataset with its publication pending |
Keyword List
curator keyword: Biological |
submitter keyword: Primary Ciliary Dyskinesia |
Contact List
Alex von Kriegsheim |
contact affiliation | University of Edinburgh |
contact email | alex.vonkriegsheim@igmm.ed.ac.uk |
lab head | |
Alex von kriegsheim |
contact affiliation | University of Edinburgh |
contact email | alex.vonkriegsheim@ucd.ie |
dataset submitter | |
Full Dataset Link List
Dataset FTP location
NOTE: Most web browsers have now discontinued native support for FTP access within the browser window. But you can usually install another FTP app (we recommend FileZilla) and configure your browser to launch the external application when you click on this FTP link. Or otherwise, launch an app that supports FTP (like FileZilla) and use this address: ftp://ftp.pride.ebi.ac.uk/pride/data/archive/2018/07/PXD006849 |
PRIDE project URI |
Repository Record List
[ + ]
[ - ]
- PRIDE
- PXD006849
- Label: PRIDE project
- Name: ZMYND10 functions in a chaperone-relay during axonemal dynein assembly