PXD024478

PXD024478 is an original dataset announced via ProteomeXchange.

Title	RNA polymerase I transcription disturbances lead to instable ribosomes in Cockayne syndrome
Description	DNA-repair factors of the Nucleotide -Excision-Repair (NER) pathway are part of the basal transcription apparatus of RNA polymerase I. Mutations in these factors can give rise to developmental disorders with symptoms that are typical for the aging body. Here we show that in Cockayne syndrome (CS) RNA polymerase I transcription elongation and the processing of the pre-rRNA are disturbed. The mature 18S rRNA is reduced and isolated ribosomes lack specific ribosomal proteins. Ribosomal proteins are susceptible to unfolding and the proteome of CS cells is heat-sensitive, indicating misfolded proteins and an error prone translation process in CS cells. Pharmaceutical chaperones restored impaired cellular proliferation. Thus, we provide evidence for severe malfunction in protein synthesis which together with a loss of proteostasis constitute the underlying pathophysiology in CS.
HostingRepository	PRIDE
AnnounceDate	2021-11-16
AnnouncementXML	Submission_2021-11-16_06:45:03.351.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Christina Ludwig
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	iodoacetamide derivatized residue
Instrument	Q Exactive HF-X

Revision	Datetime	Status	ChangeLog Entry
0	2021-03-02 22:53:53	ID requested
⏵ 1	2021-11-16 06:45:04	announced

Qiang M, Khalid F, Phan T, Ludwig C, Scharffetter-Kochanek K, Iben S, Cockayne Syndrome-Associated CSA and CSB Mutations Impair Ribosome Biogenesis, Ribosomal Protein Stability, and Global Protein Folding. Cells, 10(7):(2021) [pubmed]

submitter keyword: RNA polymerase I, ribosome, Cockayne syndrome, translational fidelity, loss of proteostasis

Christina Ludwig
contact affiliation	Bavarian Center for Biomolecular Mass Spectrometry (BayBioMS), Technical University of Munich (TUM), Freising, Germany
contact email	tina.ludwig@tum.de
lab head
Christina Ludwig
contact affiliation	TU Munich
contact email	tina.ludwig@tum.de
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD024478
     1. Label: PRIDE project
     2. Name: RNA polymerase I transcription disturbances lead to instable ribosomes in Cockayne syndrome