title: Identification and characterization of mouse MTO1 gene related to mitochondrial tRNA modification.
abstract: The nucleotide modification in tRNA plays a pivotal role in the fidelity of translational process. The defects in nucleotide modification have often been observed in the mutated mitochondrial tRNAs associated with human diseases. Recently, MTO1-like protein in bacteria and yeast has been implicated to be a component of tRNA modification pathway. Here we report the identification and characterization of mouse MTO1 homolog. The mouse MTO1 gene containing 12 exons encodes a 669-residue protein with a strong homology to the MTO1-like proteins of bacteria and yeast, related to tRNA modification. Functional conservation of this protein is supported by the observation that the isolated mouse MTO1 cDNA can complement the respiratory-deficient phenotype of yeast mto1 cells carrying P(R)(454) mutation. MTO1 is ubiquitously expressed in various tissues, but with markedly elevated expression in tissues of high metabolic rates. Furthermore, we showed that mouse Mto1 localizes in mitochondrion. These observations suggest that the mouse MTO1 is a structural and functional homolog of yeast MTO1, thereby playing a role in the mitochondrial tRNA modification and protein synthesis.
authors.affiliation: Division and Program in Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.
authors.forename: Ronghua
authors.initials: R
authors.lastname: Li
authors.forename: Xiaoming
authors.initials: X
authors.lastname: Li
authors.forename: Qinfeng
authors.initials: Q
authors.lastname: Yan
authors.forename: Jun
authors.initials: J
authors.lastname: Qin Mo
authors.forename: Min-Xin
authors.initials: MX
authors.lastname: Guan
mesh_terms: D000818:Animals; D002352:Carrier Proteins / Q000235:genetics*; D002460:Cell Line; D051379:Mice; D024101:Mitochondrial Proteins; D012313:RNA; D000077278:RNA, Mitochondrial; D012343:RNA, Transfer; D016601:RNA-Binding Proteins; D029701:Saccharomyces cerevisiae Proteins; D014162:Transfection; D015003:Yeasts / Q000235:genetics
chemical_list: D002352:Carrier Proteins; C115189:MTO1 protein, S cerevisiae; C492006:MTO1 protein, human; D024101:Mitochondrial Proteins; C492007:Mto1 protein, mouse; D000077278:RNA, Mitochondrial; D016601:RNA-Binding Proteins; D029701:Saccharomyces cerevisiae Proteins; D012313:RNA; D012343:RNA, Transfer