Proteomics

Dataset Information

0

Fe-S clusters-binding proteins in mitoribosome

ABSTRACT: 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.

INSTRUMENT(S): Orbitrap Fusion Lumos

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Cell Culture

SUBMITTER: Hui Zhong  

LAB HEAD: Antoni Barrientos

PROVIDER: PXD039321 | Pride | 2024-01-26

REPOSITORIES: Pride

Json Xml

Dataset's files

Source:
Action DRS
MS222045_AB_HZ.pdStudy Other
MS222045_AB_HZ.pdStudy.bak Other
MS222045_AB_HZ_1.sf3 Other
MS222045_AB_HZ_2ndZip_Plex_1.raw Raw
MS222045_AB_HZ_2ndZip_Plex_1_2.msf Msf
Items per page:
1 - 5 of 13
altmetric image

Publications

BOLA3 and NFU1 link mitoribosome iron-sulfur cluster assembly to multiple mitochondrial dysfunctions syndrome.

Zhong Hui H   Janer Alexandre A   Khalimonchuk Oleh O   Antonicka Hana H   Shoubridge Eric A EA   Barrientos Antoni A  

Nucleic acids research 20231101 21

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 a structural role 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 find that the mitoribosome receives its [2F  ...[more]

Similar Datasets

Transcriptomics Expression of a dominant‐negative AtNEET‐H89C protein disrupts iron–sulfur metabolism and iron homeostasis in Arabidopsis
2019-12-22 | GSE127300 | GEO
Proteomics FeS protein levels in Arabidopsis plants expressing a mutated NEET protein
2021-01-24 | PXD015447 | panorama
Proteomics The plastidial Arabidopsis thaliana NFU1 delivers [4Fe-4S] clusters to specific client proteins
2020-07-06 | PXD015295 | Pride
Proteomics METTL17 is an Fe-S cluster checkpoint for mitochondrial translation
2023-12-27 | PXD045443 | Pride
Metabolomics A pathogenic role for histone H3 copper reductase activity in a yeast model of Friedreich’s Ataxia
2021-10-21 | ST001954 | MetabolomicsWorkbench
Proteomics AIM32 is a dual-localized 2Fe-2S mitochondrial protein that functions in redox quality control
2022-02-16 | PXD021662 | Pride
Transcriptomics Interaction between mitoNEET and NAF-1 in cancer cells
2017-03-25 | GSE87626 | GEO
Proteomics Incomplete cytosolic iron-sulfur cluster assembly machinery of Giardia intestinalis is partially associated with mitosomes
2016-09-29 | PXD004722 | Pride
Other Regulators of mitonuclear balance link mitochondrial metabolism to mtDNA expression
2023-08-17 | GSE224818 | GEO
Other Regulators of mitonuclear balance link mitochondrial metabolism to mtDNA expression
2023-08-17 | GSE224817 | GEO