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PXD006849 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleZMYND10 functions in a chaperone-relay during axonemal dynein assembly
DescriptionMolecular 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.
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterAlex von kriegsheim
SpeciesList scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationListNo PTMs are included in the dataset
InstrumentQ Exactive
Dataset History
RevisionDatetimeStatusChangeLog Entry
02017-06-30 05:27:32ID requested
12018-07-12 06:36:54announced
Publication List
Dataset with its publication pending
Keyword List
curator keyword: Biological
submitter keyword: Primary Ciliary Dyskinesia
Contact List
Alex von Kriegsheim
contact affiliationUniversity of Edinburgh
contact emailalex.vonkriegsheim@igmm.ed.ac.uk
lab head
Alex von kriegsheim
contact affiliationSystems Biology
contact emailalex.vonkriegsheim@ucd.ie
dataset submitter
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Dataset FTP location
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