PXD056042

PXD056042 is an original dataset announced via ProteomeXchange.

Title	NBS1 facilitates pre-ribosomal RNA biogenesis
Description	Mutations of NBS1 gene result in Nijmegen breakage syndrome (NBS), and the gene encodes NBS1 that forms a complex with MRE11 and RAD50 and participates in DNA damage repair. However, the molecular mechanism by which the mutations of NBS1 cause clinical phenotypes of NBS, such as craniofacial dysmorphism, is still unclear. Here, we show that NBS1 localizes at the rDNA loci in the nucleoli and interacts with ribosome RNA (rRNA) transcription machinery including RNA polymerase I (Pol I) and TCOF1. Loss of NBS1 impairs Pol I-dependent transcription of pre-rRNA and induces nucleolar stress. In particular, lacking Nbs1 in mouse neural crest cells not only leads to the reduction of ribosome biogenesis but also craniofacial abnormalities during prenatal development. Moreover, the C-terminus of NBS1 is associated with pre-rRNA and a number of pre-rRNA processing factors, which may also facilitate pre-rRNA maturation. Taken together, our study reveals the functions of NBS1 in rRNA biogenesis.
HostingRepository	PRIDE
AnnounceDate	2025-09-15
AnnouncementXML	Submission_2025-09-14_16:20:10.667.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Man Luo
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	biotinylated residue
Instrument	LCQ Classic; Q Exactive

Revision	Datetime	Status	ChangeLog Entry
0	2024-09-20 01:06:49	ID requested
⏵ 1	2025-09-14 16:20:11	announced

10.1073/pnas.2422029122;

Luo M, Yu X, NBS1 facilitates preribosomal RNA biogenesis. Proc Natl Acad Sci U S A, 122(11):e2422029122(2025) [pubmed]

submitter keyword: Pre-ribosomal RNA, NBS1, nucleolus

Xiaochun Yu
contact affiliation	Westlake University 600 Dunyu, Hangzhou Zhejiang, 310030, China
contact email	yuxiaochun@westlake.edu.cn
lab head
Man Luo
contact affiliation	Westlake University
contact email	luoman@westlake.edu.cn
dataset submitter

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/2025/09/PXD056042

PRIDE project URI

• PRIDE
  1. PXD056042
     1. Label: PRIDE project
     2. Name: NBS1 facilitates pre-ribosomal RNA biogenesis