The 2-thiouridylase function of the human MTU1 (TRMU) enzyme is dispensable for mitochondrial translation.
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Nucleoside modifications in the regulation of gene expression: focus on tRNAModification of the wobble uridine in bacterial and mitochondrial tRNAs reading NNA/NNG triplets of 2-codon boxesMtu1-Mediated Thiouridine Formation of Mitochondrial tRNAs Is Required for Mitochondrial Translation and Is Involved in Reversible Infantile Liver InjuryMutation in MRPS34 compromises protein synthesis and causes mitochondrial dysfunctionReduced TRMU expression increases the sensitivity of hair-cell-like HEI-OC-1 cells to neomycin damage in vitroMTO1 mutations are associated with hypertrophic cardiomyopathy and lactic acidosis and cause respiratory chain deficiency in humans and yeast.Nuclear modifier MTO2 modulates the aminoglycoside-sensitivity of mitochondrial 15S rRNA C1477G mutation in Saccharomyces cerevisiae.MTO1 mediates tissue specificity of OXPHOS defects via tRNA modification and translation optimization, which can be bypassed by dietary intervention.Hepatic Copper Accumulation: A Novel Feature in Transient Infantile Liver Failure Due to TRMU Mutations?Defective Expression of the Mitochondrial-tRNA Modifying Enzyme GTPBP3 Triggers AMPK-Mediated Adaptive Responses Involving Complex I Assembly Factors, Uncoupling Protein 2, and the Mitochondrial Pyruvate Carrier.Transfer RNA modifications: nature's combinatorial chemistry playground.The output of the tRNA modification pathways controlled by the Escherichia coli MnmEG and MnmC enzymes depends on the growth conditions and the tRNA species.Mitochondria: impaired mitochondrial translation in human diseaseReversible infantile mitochondrial diseases.Nuclear-encoded factors involved in post-transcriptional processing and modification of mitochondrial tRNAs in human disease.microRNA-mediated differential expression of TRMU, GTPBP3 and MTO1 in cell models of mitochondrial-DNA diseases.Mutations in the Caenorhabditis elegans orthologs of human genes required for mitochondrial tRNA modification cause similar electron transport chain defects but different nuclear responses.Altered 2-thiouridylation impairs mitochondrial translation in reversible infantile respiratory chain deficiency.Biochemical Evidence for a Nuclear Modifier Allele (A10S) in TRMU (Methylaminomethyl-2-thiouridylate-methyltransferase) Related to Mitochondrial tRNA Modification in the Phenotypic Manifestation of Deafness-associated 12S rRNA Mutation.Mitochondrial Infantile Liver Disease due to TRMU Gene Mutations: Three New Cases.Mutations of the mitochondrial-tRNA modifier MTO1 cause hypertrophic cardiomyopathy and lactic acidosis.A gene encoding a DUF523 domain protein is involved in the conversion of 2-thiouracil into uracil.The genotypic and phenotypic spectrum of MTO1 deficiency.Defects in the mitochondrial-tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPARγ-UCP2-AMPK axis.The role of mitochondrial tRNAPhe C628T variant in deafness expression.Cysteine Supplementation May be Beneficial in a Subgroup of Mitochondrial Translation Deficiencies.TRMU (MTO2; MTU1) transfers a sulfur atom to 5-taurinomethyluridine-34 in tRNARNA modification landscape of the human mitochondrial tRNA regulates protein synthesisA novel mutation in YARS2 causes myopathy with lactic acidosis and sideroblastic anemia
P2860
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P2860
The 2-thiouridylase function of the human MTU1 (TRMU) enzyme is dispensable for mitochondrial translation.
description
2011 nî lūn-bûn
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2011年の論文
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2011年学术文章
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2011年学术文章
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2011年学术文章
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2011年学术文章
@zh-hans
2011年学术文章
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2011年学术文章
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2011年學術文章
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name
The 2-thiouridylase function o ...... for mitochondrial translation.
@en
The 2-thiouridylase function of the human MTU1
@nl
type
label
The 2-thiouridylase function o ...... for mitochondrial translation.
@en
The 2-thiouridylase function of the human MTU1
@nl
prefLabel
The 2-thiouridylase function o ...... for mitochondrial translation.
@en
The 2-thiouridylase function of the human MTU1
@nl
P2860
P356
P1476
The 2-thiouridylase function o ...... for mitochondrial translation.
@en
P2093
Eric A Shoubridge
Florin Sasarman
P2860
P304
P356
10.1093/HMG/DDR397
P577
2011-09-01T00:00:00Z