PXD054386-1

PXD054386 is an original dataset announced via ProteomeXchange.

Title	Mitochondrial translation regulates terminal erythroid differentiation by maintaining iron homeostasis
Description	Mitochondrial tRNA taurine modifications mediated by mitochondrial tRNA translation optimization 1 (Mto1) is essential for the mitochondrial protein translation. Mto1 deficiency was shown to induce proteostress in embryonic stem cells. Recently a patient with MTO1 gene mutation presented with severe anemia was reported, which led us to hypothesize that Mto1 dysfunctions may result in defective erythropoiesis. Hematopoietic-specific Mto1 conditional knockout (cKO) mice were embryonic lethal due to niche-independent defective terminal erythroid differentiation. Mechanistically, mitochondrial oxidative phosphorylation complexes were severely impaired in the Mto1 cKO fetal liver and this was followed by cytoplasmic iron accumulation. Overloaded cytoplasmic iron promoted heme biosynthesis, which induced an unfolded protein response via the IRE1-Xbp1 signaling pathway in Mto1 cKO erythroblasts. An iron chelator rescued erythroid terminal differentiation in the Mto1 cKO fetal liver in vitro. This novel non-energy-related mitochondrial iron homeostasis revealed the indispensable role of mitochondrial tRNA modification in hematopoiesis.
HostingRepository	PRIDE
AnnounceDate	2025-01-20
AnnouncementXML	Submission_2025-01-20_03:08:36.750.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Michal Levin
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	acetylated residue; monohydroxylated residue
Instrument	Orbitrap Exploris 480

Revision	Datetime	Status	ChangeLog Entry
0	2024-07-30 05:15:20	ID requested
⏵ 1	2025-01-20 03:08:37	announced

Dataset with its publication pending

submitter keyword: LC-MS/MS

Falk Butter
contact affiliation	Federal Research Institute for Animal Health Germany
contact email	Falk.Butter@fli.d
lab head
Michal Levin
contact affiliation	Institute of Molecular Biology
contact email	m.levin@imb-mainz.de
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD054386
     1. Label: PRIDE project
     2. Name: Mitochondrial translation regulates terminal erythroid differentiation by maintaining iron homeostasis