PXD039321

PXD039321 is an original dataset announced via ProteomeXchange.

Title	Fe-S clusters-binding proteins in mitoribosome
Description	The human mitochondrial ribosome contains three [2Fe-2S] clusters whose assembly pathway, role, and implications for mitochondrial and metabolic diseases are unknown. Here, structure-function correlation studies show that the clusters play structural roles during mitoribosome assembly. To uncover the assembly pathway, we have examined the effect of silencing the expression of Fe-S cluster biosynthetic and delivery factors on mitoribosome stability. We have found that the mitoribosome receives its [2Fe-2S] clusters from the GLRX5-BOLA3. Additionally, the assembly of the small subunit depends on METTL17, recently reported to contain a [4Fe-4S] cluster, which we found to be inserted via ISCA1-NFU1. Consistently, fibroblasts from patients suffering from “multiple mitochondrial dysfunction” syndrome due to mutations in BOLA3 or NFU1 display previously unrecognized attenuation of mitochondrial protein synthesis that contributes to their cellular and pathophysiological phenotypes. Finally, we report that, in addition to their structural role, the mitoribosomal [2Fe-2S] clusters sense changes in the redox environment. In this way, the mitoribosome checks the availability and stability of mitochondrial Fe-S clusters to regulate organellar protein synthesis accordingly.
HostingRepository	PRIDE
AnnounceDate	2024-10-22
AnnouncementXML	Submission_2024-10-22_06:25:51.021.xml
DigitalObjectIdentifier	https://dx.doi.org/10.6019/PXD039321
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Supported dataset by repository
PrimarySubmitter	Hui Zhong
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	No PTMs are included in the dataset
Instrument	Orbitrap Fusion Lumos

Revision	Datetime	Status	ChangeLog Entry
0	2023-01-09 02:11:17	ID requested
1	2024-01-26 07:10:41	announced
⏵ 2	2024-10-22 06:25:52	announced	2024-10-22: Updated project metadata.

10.1093/nar/gkad842;

Zhong H, Janer A, Khalimonchuk O, Antonicka H, Shoubridge EA, Barrientos A, BOLA3 and NFU1 link mitoribosome iron-sulfur cluster assembly to multiple mitochondrial dysfunctions syndrome. Nucleic Acids Res, 51(21):11797-11812(2023) [pubmed]

10.6019/PXD039321;

submitter keyword: Fe-S cluster,Mitoribosome

Antoni Barrientos
contact affiliation	Department of Neurology, Miller School of Medicine, University of Miami, Miami, FL, USA
contact email	abarrientos@med.miami.edu
lab head
Hui Zhong
contact affiliation	University of Miami
contact email	hxz213@miami.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/2024/01/PXD039321

PRIDE project URI

• PRIDE
  1. PXD039321
     1. Label: PRIDE project
     2. Name: Fe-S clusters-binding proteins in mitoribosome