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MTO1 cystathionine gamma-synthase

mto1, cystathionine gamma-synthase, CGS1, mod20, mbo1
encodes a cystathionine gamma-synthase, which performs the first committed step in methionine biosynthesis. A conserved motif of 13 amino acids in the first exon is required for posttranscriptional autoregulation. This enzyme shares the same substrate as threonine synthase (TS) and its absence transcriptionally affects 8 genes in the genome. (from NCBI)
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Top mentioned proteins: ACID, CAN, STEP, HAD, V1a
Papers on mto1
Optic neuropathy, cardiomyopathy, cognitive disability in patients with a homozygous mutation in the nuclear MTO1 and a mitochondrial MT-TF variant.
Hamel et al., Montpellier, France. In Am J Med Genet A, Oct 2015
Mitochondrial DNA and exome sequencing revealed a novel homozygous mutation in the nuclear MTO1 gene and the homoplasmic m.593T>G mutation in the mitochondrial MT-TF gene.
MTO1 mediates tissue specificity of OXPHOS defects via tRNA modification and translation optimization, which can be bypassed by dietary intervention.
Wenz et al., Köln, Germany. In Hum Mol Genet, May 2015
Here we present regulation of mitochondrial translation by the Mitochondrial Translation Optimization Factor 1, MTO1, as a novel player in this scenario.
The ROS-sensitive microRNA-9/9* controls the expression of mitochondrial tRNA-modifying enzymes and is involved in the molecular mechanism of MELAS syndrome.
Armengod et al., Valencia, Spain. In Hum Mol Genet, 2015
We demonstrated that oxidative stress mediates an NFkB-dependent induction of microRNA-9/9*, which acts as a post-transcriptional negative regulator of the mt-tRNA-modification enzymes GTPBP3, MTO1 and TRMU.
Mutations in GTPBP3 cause a mitochondrial translation defect associated with hypertrophic cardiomyopathy, lactic acidosis, and encephalopathy.
Prokisch et al., München, Germany. In Am J Hum Genet, 2015
In contrast to individuals with mutations in MTO1, the protein product of which is predicted to participate in the generation of the same modification, most individuals with GTPBP3 mutations developed neurological symptoms and MRI involvement of thalamus, putamen, and brainstem resembling Leigh syndrome.
MTO1 worked as a modifier in the aminoglycosides sensitivity of yeast carrying a mitochondrial 15S rRNA C1477G mutation.
Yan et al., Hangzhou, China. In Plos One, 2014
MTO1, together with MSS1 and MTO2, is a gene involved in the pathway of encoding a mitochondria-specific RNA-modifying enzyme related to the post-transcriptional modification of mitochondrial tRNAs.
The N-terminal cleavable pre-sequence encoded in the first exon of cystathionine γ-synthase contains two different functional domains for chloroplast targeting and regulation of gene expression.
Naito et al., Tokyo, Japan. In Plant Cell Physiol, 2014
This N-terminal extension contains the cTP and several functional domains including an MTO1 region, the cis-element for post-transcriptional feedback regulation of CGS1 that codes for CGS.
Use of whole-exome sequencing to determine the genetic basis of multiple mitochondrial respiratory chain complex deficiencies.
Chinnery et al., Newcastle upon Tyne, United Kingdom. In Jama, 2014
These included recurrent mutations in RMND1, AARS2, and MTO1, each on a haplotype background consistent with a shared founder allele, and potential novel mutations in 4 possible mitochondrial disease genes (VARS2, GARS, FLAD1, and PTCD1).
Ribosomes in a stacked array: elucidation of the step in translation elongation at which they are stalled during S-adenosyl-L-methionine-induced translation arrest of CGS1 mRNA.
Naito et al., Sapporo, Japan. In J Biol Chem, 2014
Expression of CGS1, which codes for an enzyme of methionine biosynthesis, is feedback-regulated by mRNA degradation in response to S-adenosyl-L-methionine (AdoMet).
Dynamics of cell shape inheritance in fission yeast.
Carazo-Salas et al., Cambridge, United Kingdom. In Plos One, 2013
Finally, we find that one of those pathways corresponds to the swc2-swr1-vps71 SWR1/SRCAP chromatin remodelling complex, which acts additively to the known mal3-tip1-mto1-mto2 microtubule and tea1-tea2-tea4-pom1 polarity machineries.
MTO1-deficient mouse model mirrors the human phenotype showing complex I defect and cardiomyopathy.
Klopstock et al., München, Germany. In Plos One, 2013
Recently, mutations in the mitochondrial translation optimization factor 1 gene (MTO1) were identified as causative in children with hypertrophic cardiomyopathy, lactic acidosis and respiratory chain defect.
Nuclear factors: roles related to mitochondrial deafness.
Yang et al., Hangzhou, China. In Gene, 2013
TFB1M, MTO1, GTPBP3, and TRMU are modifier genes.
A halt in poly(A) shortening during S-adenosyl-L-methionine-induced translation arrest in CGS1 mRNA of Arabidopsis thaliana.
Naito et al., Japan. In Genes Genet Syst, 2012
Cystathionine γ-synthase (CGS) catalyzes the first committed step of methionine (Met) biosynthesis in plants.
Mutations of the mitochondrial-tRNA modifier MTO1 cause hypertrophic cardiomyopathy and lactic acidosis.
Zeviani et al., Milano, Italy. In Am J Hum Genet, 2012
The respiratory yeast phenotype for MTO1 was dramatically worsened in stress conditions and in the presence of a paromomycin-resistant (P(R)) mitochondrial rRNA mutation
Mutation in MTO1 involved in tRNA modification impairs mitochondrial RNA metabolism in the yeast Saccharomyces cerevisiae.
Guan et al., Cincinnati, United States. In Mitochondrion, 2009
These data strongly indicate that unmodified tRNA caused by the deletion of MTO1 gene caused the instability of mitochondrial tRNAs and mRNAs and an impairment of aminoacylation of mitochondrial tRNAs.
Nascent peptide-mediated translation elongation arrest of Arabidopsis thaliana CGS1 mRNA occurs autonomously.
Naito et al., Sapporo, Japan. In Plant Cell Physiol, 2008
CGS1 mRNA degradation involves a plant-specific mechanism.
Ribosome stacking defines CGS1 mRNA degradation sites during nascent peptide-mediated translation arrest.
Naito et al., Sapporo, Japan. In Plant Cell Physiol, 2008
These results show that S-adenosyl-L-methionine induces the stacking of ribosomes on CGS1 mRNA and that multiple mRNA degradation sites probably correspond to each stacked ribosome.
Molecular characterization of putative modulatory factors in two Spanish families with A1555G deafness.
López de Munain et al., San Sebastián, Spain. In Audiol Neurootol, 2007
proposed linkage in chromosome 8 and the association with TRMU and MTO1 genes were studied in A1555G deafness
Self-organization of interphase microtubule arrays in fission yeast.
Nurse et al., London, United Kingdom. In Nat Cell Biol, 2006
By artificially enucleating cells, we show that arrays can form de novo (self-organize) without nuclear-associated MTOCs, but require the microtubule nucleator mod20-mbo1-mto1 (refs 3-5), the bundling factor ase1 (refs 6,7), and the kinesin klp2 (refs 8,9).
Engineering of cysteine and methionine biosynthesis in potato.
Hesse et al., Germany. In Amino Acids, 2001
From the data presented here and in previous work we conclude that threonine synthase and not cystathionine gamma-synthase as expected from studies of Arabidopsis constitutes the main regulatory control point of methionine synthesis in potato.
Evidence for autoregulation of cystathionine gamma-synthase mRNA stability in Arabidopsis.
Naito et al., Sapporo, Japan. In Science, 1999
Analysis of Arabidopsis mutants that overaccumulate soluble methionine (Met) revealed that the gene for cystathionine gamma-synthase (CGS), the key enzyme in Met biosynthesis, is regulated at the level of mRNA stability.
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