PXD065571-1

PXD065571 is an original dataset announced via ProteomeXchange.

Title	C18orf21/RMP24 and Nepro/RMP64 are RNase MRP-specific subunits required for 40S ribosome biogenesis
Description	RNase MRP and RNase P are evolutionarily related complexes that facilitate rRNA and tRNA biogenesis, respectively. The two enzymes share nearly all protein subunits and have evolutionarily related catalytic RNAs. Notably, RNase P includes a unique subunit, RPP21, whereas no RNase MRP-specific proteins have been found in humans, limiting molecular analyses of RNase MRP function. Here, we identify the RNase MRP-specific proteins, C18orf21/RMP24 and NEPRO/RMP64. C18orf21/RMP24 and RPP21 display significant structural homology, but we identify specific regions that drive interactions with their respective complexes. By targeting these RNase MRP-specific subunits, our functional analysis reveals that RNase MRP is essential rRNA processing and preferentially required for 40S ribosome biogenesis. Finally, we determine that disease-associated mutations in RMP64 impair its association with RNase MRP subunits. Together, our findings elucidate the molecular determinants of RNase MRP function and underscore its critical role in ribosome biogenesis and disease.
HostingRepository	PRIDE
AnnounceDate	2025-10-23
AnnouncementXML	Submission_2025-10-23_06:57:44.805.xml
DigitalObjectIdentifier	https://dx.doi.org/10.6019/PXD065571
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Supported dataset by repository
PrimarySubmitter	Eric Smith
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: NEWT:9606;
ModificationList	phosphorylated residue; L-methionine removal; monohydroxylated residue; iodoacetamide derivatized residue
Instrument	Orbitrap Exploris 480

Revision	Datetime	Status	ChangeLog Entry
0	2025-06-27 13:08:30	ID requested
⏵ 1	2025-10-23 06:57:45	announced

10.1038/S41594-025-01690-7;

10.6019/PXD065571;

submitter keyword: RNase P,RNase MRP, rRNA biogenesis

Iain Cheeseman
contact affiliation	Whitehead Institute for Biomedical Research and Department of Biology, Massachusetts Institute of Technology
contact email	icheese@wi.mit.edu
lab head
Eric Smith
contact affiliation	Whitehead Institute for Biomedical Research
contact email	ericms@wi.mit.edu
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/2025/10/PXD065571

PRIDE project URI

• PRIDE
  1. PXD065571
     1. Label: PRIDE project
     2. Name: C18orf21/RMP24 and Nepro/RMP64 are RNase MRP-specific subunits required for 40S ribosome biogenesis