Isolation and characterization of the putative nuclear modifier gene MTO1 involved in the pathogenesis of deafness-associated mitochondrial 12 S rRNA A1555G mutation
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The role of the LRPPRC (leucine-rich pentatricopeptide repeat cassette) gene in cytochrome oxidase assembly: mutation causes lowered levels of COX (cytochrome c oxidase) I and COX III mRNAA human mitochondrial GTP binding protein related to tRNA modification may modulate phenotypic expression of the deafness-associated mitochondrial 12S rRNA mutationMutations in MTO2 related to tRNA modification impair mitochondrial gene expression and protein synthesis in the presence of a paromomycin resistance mutation in mitochondrial 15 S rRNAMutation in TRMU related to transfer RNA modification modulates the phenotypic expression of the deafness-associated mitochondrial 12S ribosomal RNA mutationsModification of the wobble uridine in bacterial and mitochondrial tRNAs reading NNA/NNG triplets of 2-codon boxesThe human mitochondrial transcriptome and the RNA-binding proteins that regulate its expressionKissing G domains of MnmE monitored by X-ray crystallography and pulse electron paramagnetic resonance spectroscopyA peep into mitochondrial disorder: multifaceted from mitochondrial DNA mutations to nuclear gene modulationFurther insights into the tRNA modification process controlled by proteins MnmE and GidA of Escherichia coliMaternally inherited aminoglycoside-induced and nonsyndromic deafness is associated with the novel C1494T mutation in the mitochondrial 12S rRNA gene in a large Chinese familyA complete landscape of post-transcriptional modifications in mammalian mitochondrial tRNAs.Human mitochondrial leucyl-tRNA synthetase corrects mitochondrial dysfunctions due to the tRNALeu(UUR) A3243G mutation, associated with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like symptoms and diabetesMutations in GTPBP3 cause a mitochondrial translation defect associated with hypertrophic cardiomyopathy, lactic acidosis, and encephalopathytRNA modification enzymes GidA and MnmE: potential role in virulence of bacterial pathogens.Characterization of human GTPBP3, a GTP-binding protein involved in mitochondrial tRNA modification.Nuclear modifier MTO2 modulates the aminoglycoside-sensitivity of mitochondrial 15S rRNA C1477G mutation in Saccharomyces cerevisiae.Mechanistic study on the nuclear modifier gene MSS1 mutation suppressing neomycin sensitivity of the mitochondrial 15S rRNA C1477G mutation in Saccharomyces cerevisiaeDisorders of mitochondrial protein synthesis.Taurine-containing uridine modifications in tRNA anticodons are required to decipher non-universal genetic codes in ascidian mitochondria.MTO1 worked as a modifier in the aminoglycosides sensitivity of yeast carrying a mitochondrial 15S rRNA C1477G mutationMitochondrial transcript maturation and its disordersMitochondrial ribosome assembly in health and diseaseMitochondrial deafness alleles confer misreading of the genetic codeExpression and maintenance of mitochondrial DNA: new insights into human disease pathologyMitochondrial m.1584A 12S m62A rRNA methylation in families with m.1555A>G associated hearing loss.Are GJB2 mutations an aggravating factor in the phenotypic expression of mitochondrial non-syndromic deafness?Nuclear-encoded factors involved in post-transcriptional processing and modification of mitochondrial tRNAs in human disease.Invited review: MnmE, a GTPase that drives a complex tRNA modification reaction.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.Stabilization of G domain conformations in the tRNA-modifying MnmE-GidA complex observed with double electron electron resonance spectroscopy.The structure of the TrmE GTP-binding protein and its implications for tRNA modification.Dimerisation-dependent GTPase reaction of MnmE: how potassium acts as GTPase-activating element.The tRNA-modifying function of MnmE is controlled by post-hydrolysis steps of its GTPase cycle.Mutations of the mitochondrial-tRNA modifier MTO1 cause hypertrophic cardiomyopathy and lactic acidosis.Mutation in MTO1 involved in tRNA modification impairs mitochondrial RNA metabolism in the yeast Saccharomyces cerevisiaeLack of a modulative factor in locus 8p23 in a Finnish family with nonsyndromic sensorineural hearing loss associated with the 1555A>G mitochondrial DNA mutation.Evolutionarily conserved proteins MnmE and GidA catalyze the formation of two methyluridine derivatives at tRNA wobble positions.Mutations in mitochondrial ribosomal protein MRPL12 leads to growth retardation, neurological deterioration and mitochondrial translation deficiency.The GTPase activity and C-terminal cysteine of the Escherichia coli MnmE protein are essential for its tRNA modifying function.Effects of mutagenesis in the switch I region and conserved arginines of Escherichia coli MnmE protein, a GTPase involved in tRNA modification.
P2860
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P2860
Isolation and characterization of the putative nuclear modifier gene MTO1 involved in the pathogenesis of deafness-associated mitochondrial 12 S rRNA A1555G mutation
description
2002 nî lūn-bûn
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2002 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
2002 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
2002年の論文
@ja
2002年論文
@yue
2002年論文
@zh-hant
2002年論文
@zh-hk
2002年論文
@zh-mo
2002年論文
@zh-tw
2002年论文
@wuu
name
Isolation and characterization ...... rial 12 S rRNA A1555G mutation
@ast
Isolation and characterization ...... rial 12 S rRNA A1555G mutation
@en
Isolation and characterization ...... rial 12 S rRNA A1555G mutation
@nl
type
label
Isolation and characterization ...... rial 12 S rRNA A1555G mutation
@ast
Isolation and characterization ...... rial 12 S rRNA A1555G mutation
@en
Isolation and characterization ...... rial 12 S rRNA A1555G mutation
@nl
prefLabel
Isolation and characterization ...... rial 12 S rRNA A1555G mutation
@ast
Isolation and characterization ...... rial 12 S rRNA A1555G mutation
@en
Isolation and characterization ...... rial 12 S rRNA A1555G mutation
@nl
P2093
P2860
P356
P1476
Isolation and characterization ...... rial 12 S rRNA A1555G mutation
@en
P2093
Min-Xin Guan
Ronghua Li
Xiaoming Li
Xinhua Lin
P2860
P304
P356
10.1074/JBC.M203267200
P407
P577
2002-07-26T00:00:00Z