| 1305367 | Gtpbp3 | GTP binding protein 3 | ENCODES a protein that exhibits GTPase activity (ortholog); tRNA 5-taurinomethyluridine synthase activity (ortholog); INVOLVED IN mitochondrial tRNA wobble uridine modification (ortholog); ASSOCIATED WITH combined oxidative phosphorylation deficiency 23 (ortholog); genetic disease (ortholog); hypert rophic cardiomyopathy (ortholog); FOUND IN cytoplasm (ortholog); GTPBP3-MTO1 complex (ortholog); mitochondrion (ortholog); INTERACTS WITH 2,3,7,8-tetrachlorodibenzodioxine; 2,3,7,8-Tetrachlorodibenzofuran; 3-chloropropane-1,2-diol | 16 | 18209747 | 18214838 | Rat | 86 | symbol , old_gene_name , PhenoGen , description | gene, protein-coding, PROVISIONAL [RefSeq] |
| 1313350 | GTPBP3 | GTP binding protein 3, mitochondrial | This locus encodes a GTP-binding protein. The encoded protein is localized to the mitochondria and may play a role in mitochondrial tRNA modification. Polymorphisms at this locus may be associated with severity of aminoglycoside-induced deafness, a disease associated with a mutation in the 12S rRNA. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Sep 2010] | 19 | 17334982 | 17342731 | Human | 143 | symbol , old_gene_name , COSMIC , Human Proteome Map | gene, protein-coding, REVIEWED [RefSeq] |
| 1313351 | Gtpbp3 | GTP binding protein 3 | Predicted to enable GTP binding activity and GTPase activity. Predicted to be involved in tRNA methylation and tRNA wobble uridine modification. Located in mitochondrion. Human ortholog(s) of this gene implicated in combined oxidative phosphorylation deficiency 23. Orthologous to human GTPBP3 font-weight:700;'>GTPBP3 (GTP binding protein 3, mitochondrial). [provided by Alliance of Genome Resources, Apr 2025] | 8 | 71938788 | 71952268 | Mouse | 99 | symbol , old_gene_name , PhenoGen , description | gene, protein-coding, VALIDATED [RefSeq] |
| 8977287 | Gtpbp3 | GTP binding protein 3, mitochondrial | ENCODES a protein that exhibits GTPase activity (ortholog); tRNA 5-taurinomethyluridine synthase activity (ortholog); INVOLVED IN mitochondrial tRNA wobble uridine modification (ortholog); ASSOCIATED WITH combined oxidative phosphorylation deficiency 23 (ortholog); genetic disease (ortholog); hypert rophic cardiomyopathy (ortholog); FOUND IN cytoplasm (ortholog); GTPBP3-MTO1 complex (ortholog); mitochondrion (ortholog) | NW_004955524 | 899475 | 903168 | Chinchilla | 18 | symbol , old_gene_name , description | gene, protein-coding, MODEL [RefSeq] |
| 12044383 | GTPBP3 | GTP binding protein 3, mitochondrial | ENCODES a protein that exhibits GTP binding (inferred); GTPase activity (inferred); hydrolase activity (inferred); INVOLVED IN mitochondrial tRNA modification (inferred); mitochondrial tRNA wobble uridine modification (inferred); tRNA methylation (inferred); ASSOCIATED WITH combined oxidative phosph orylation deficiency 23 (ortholog); genetic disease (ortholog); hypertrophic cardiomyopathy (ortholog); FOUND IN nucleus (ortholog) | 19 | 16822786 | 16830787 | Bonobo | 29 | symbol , old_gene_name | gene, protein-coding, MODEL [RefSeq] |
| 12432254 | GTPBP3 | GTP binding protein 3, mitochondrial | ENCODES a protein that exhibits GTP binding (inferred); GTPase activity (inferred); hydrolase activity (inferred); INVOLVED IN mitochondrial tRNA modification (inferred); mitochondrial tRNA wobble uridine modification (inferred); tRNA modification (inferred); ASSOCIATED WITH combined oxidative phosp horylation deficiency 23 (ortholog); genetic disease (ortholog); hypertrophic cardiomyopathy (ortholog); FOUND IN cytoplasm (ortholog); nucleus (ortholog) | 20 | 45422653 | 45427434 | Dog | 30 | symbol , old_gene_name | gene, protein-coding, MODEL [RefSeq] |
| 12628265 | Gtpbp3 | GTP binding protein 3, mitochondrial | ENCODES a protein that exhibits GTP binding (inferred); GTPase activity (inferred); hydrolase activity (inferred); INVOLVED IN mitochondrial tRNA modification (inferred); mitochondrial tRNA wobble uridine modification (inferred); tRNA methylation (inferred); ASSOCIATED WITH combined oxidative phosph orylation deficiency 23 (ortholog); genetic disease (ortholog); hypertrophic cardiomyopathy (ortholog); FOUND IN nucleus (ortholog) | NW_004936596 | 3527903 | 3532931 | Squirrel | 29 | symbol , old_gene_name | gene, protein-coding, MODEL [RefSeq] |
| 18512315 | GTPBP3 | GTP binding protein 3, mitochondrial | ENCODES a protein that exhibits GTPase activity (ortholog); tRNA 5-taurinomethyluridine synthase activity (ortholog); INVOLVED IN mitochondrial tRNA wobble uridine modification (ortholog); ASSOCIATED WITH combined oxidative phosphorylation deficiency 23 (ortholog); genetic disease (ortholog); hypert rophic cardiomyopathy (ortholog); FOUND IN cytoplasm (ortholog); GTPBP3-MTO1 complex (ortholog); mitochondrion (ortholog) | | | | Green Monkey | 18 | symbol , old_gene_name , description | gene, protein-coding, MODEL [RefSeq] |
| 626140296 | Gtpbp3 | GTP binding protein 3, mitochondrial | ENCODES a protein that exhibits GTPase activity (ortholog); tRNA 5-taurinomethyluridine synthase activity (ortholog); INVOLVED IN mitochondrial tRNA wobble uridine modification (ortholog); ASSOCIATED WITH combined oxidative phosphorylation deficiency 23 (ortholog); genetic disease (ortholog); hypert rophic cardiomyopathy (ortholog); FOUND IN cytoplasm (ortholog); GTPBP3-MTO1 complex (ortholog); mitochondrion (ortholog) | | | | Black Rat | 18 | symbol , old_gene_name , description | gene, protein-coding, MODEL [RefSeq] |
| 13911085 | GTPBP3 | GTP binding protein 3, mitochondrial | ENCODES a protein that exhibits GTP binding (inferred); GTPase activity (inferred); hydrolase activity (inferred); INVOLVED IN mitochondrial tRNA modification (inferred); mitochondrial tRNA wobble uridine modification (inferred); tRNA modification (inferred); ASSOCIATED WITH combined oxidative phosp horylation deficiency 23 (ortholog); genetic disease (ortholog); hypertrophic cardiomyopathy (ortholog); FOUND IN cytoplasm (ortholog); nucleus (ortholog) | | | | Pig | 30 | symbol , old_gene_name | gene, protein-coding, MODEL [RefSeq] |
| 155234317 | gtpbp3 | | | | | | Tropical Clawed Frog | | symbol | gene, null |
| 18920772 | Gtpbp3 | GTP binding protein 3, mitochondrial | ENCODES a protein that exhibits tRNA 5-taurinomethyluridine synthase activity (ortholog); INVOLVED IN mitochondrial tRNA wobble uridine modification (ortholog); ASSOCIATED WITH combined oxidative phosphorylation deficiency 23 (ortholog); genetic disease (ortholog); hypertrophic cardiomyopathy (ortho log); FOUND IN GTPBP3-MTO1 complex (ortholog); nucleus (ortholog) | | | | Naked Mole-Rat | 28 | symbol , old_gene_name , description | gene, protein-coding, MODEL [RefSeq] |
| 155257969 | gtpbp3.S | | | | | | African Clawed Frog | | symbol | gene, null |
| 732876 | Gtpbp4 | GTP binding protein 4 | Predicted to enable several functions, including GTP binding activity; GTPase activity; and preribosome binding activity. Acts upstream of or within maturation of LSU-rRNA from tricistronic rRNA transcript (SSU-rRNA, 5.8S rRNA, LSU-rRNA). Predicted to be located in cytosol; nucleus; and perinuclear region of cytoplasm. Predicted to be active in nucleolus. Orthologous to human GTPBP4 (GTP binding protein 4). [provided by Alliance of Genome Resources, Jul 2025] | 13 | 9016039 | 9046080 | Mouse | 164 | old_gene_symbol | gene, protein-coding, VALIDATED [RefSeq] |
| 1308830 | Mto1 | mitochondrial tRNA translation optimization 1 | ENCODES a protein that exhibits tRNA 5-taurinomethyluridine synthase activity (ortholog); INVOLVED IN mitochondrial tRNA wobble uridine modification (ortholog); tRNA wobble uridine modification (ortholog); ASSOCIATED WITH combined oxidative phosphorylation deficiency (ortholog); combined oxidative p hosphorylation deficiency 10 (ortholog); Developmental Disabilities (ortholog); FOUND IN GTPBP3-MTO1 complex (ortholog); mitochondrion (ortholog); INTERACTS WITH 2,6-dinitrotoluene; 3-chloropropane-1,2-diol; 6-propyl-2-thiouracil | 8 | 88188918 | 88215516 | Rat | 75 | description | gene, protein-coding, PROVISIONAL [RefSeq] |
| 9011391 | Mto1 | mitochondrial tRNA translation optimization 1 | ENCODES a protein that exhibits tRNA 5-taurinomethyluridine synthase activity (ortholog); INVOLVED IN mitochondrial tRNA wobble uridine modification (ortholog); tRNA wobble uridine modification (ortholog); ASSOCIATED WITH combined oxidative phosphorylation deficiency (ortholog); combined oxidative p hosphorylation deficiency 10 (ortholog); Developmental Disabilities (ortholog); FOUND IN GTPBP3-MTO1 complex (ortholog); mitochondrion (ortholog) | NW_004955493 | 8636093 | 8655090 | Chinchilla | 14 | description | gene, protein-coding, MODEL [RefSeq] |
| 11835168 | MTO1 | mitochondrial tRNA translation optimization 1 | ENCODES a protein that exhibits flavin adenine dinucleotide binding (inferred); tRNA 5-taurinomethyluridine synthase activity (inferred); INVOLVED IN mitochondrial tRNA wobble uridine modification (ortholog); tRNA wobble uridine modification (ortholog); ASSOCIATED WITH combined oxidative phosphoryla tion deficiency (ortholog); combined oxidative phosphorylation deficiency 10 (ortholog); Developmental Disabilities (ortholog); FOUND IN cytoplasm (inferred); cytosol (inferred); GTPBP3-MTO1 complex (inferred) | 6 | 71282988 | 71325075 | Bonobo | 23 | description | gene, protein-coding, MODEL [RefSeq] |
| 12328719 | MTO1 | mitochondrial tRNA translation optimization 1 | ENCODES a protein that exhibits flavin adenine dinucleotide binding (inferred); tRNA 5-taurinomethyluridine synthase activity (inferred); INVOLVED IN mitochondrial tRNA wobble uridine modification (ortholog); tRNA wobble uridine modification (ortholog); ASSOCIATED WITH combined oxidative phosphoryla tion deficiency (ortholog); combined oxidative phosphorylation deficiency 10 (ortholog); Developmental Disabilities (ortholog); FOUND IN cytoplasm (inferred); GTPBP3-MTO1 complex (inferred); mitochondrion (inferred) | 12 | 35533766 | 35563058 | Dog | 22 | description | gene, protein-coding, MODEL [RefSeq] |
| 12675230 | Mto1 | mitochondrial tRNA translation optimization 1 | ENCODES a protein that exhibits flavin adenine dinucleotide binding (inferred); tRNA 5-taurinomethyluridine synthase activity (inferred); INVOLVED IN mitochondrial tRNA wobble uridine modification (ortholog); tRNA wobble uridine modification (ortholog); ASSOCIATED WITH combined oxidative phosphoryla tion deficiency (ortholog); combined oxidative phosphorylation deficiency 10 (ortholog); Developmental Disabilities (ortholog); FOUND IN cytoplasm (inferred); cytosol (inferred); GTPBP3-MTO1 complex (inferred) | NW_004936859 | 757977 | 788073 | Squirrel | 21 | description | gene, protein-coding, MODEL [RefSeq] |
| 18548691 | MTO1 | mitochondrial tRNA translation optimization 1 | ENCODES a protein that exhibits flavin adenine dinucleotide binding (inferred); tRNA 5-taurinomethyluridine synthase activity (inferred); INVOLVED IN mitochondrial tRNA wobble uridine modification (ortholog); tRNA wobble uridine modification (ortholog); ASSOCIATED WITH combined oxidative phosphoryla tion deficiency (ortholog); combined oxidative phosphorylation deficiency 10 (ortholog); Developmental Disabilities (ortholog); FOUND IN cytoplasm (inferred); cytosol (inferred); GTPBP3-MTO1 complex (inferred) | | | | Green Monkey | 21 | description | gene, protein-coding, MODEL [RefSeq] |
| 18910438 | Mto1 | mitochondrial tRNA translation optimization 1 | ENCODES a protein that exhibits tRNA 5-taurinomethyluridine synthase activity (ortholog); INVOLVED IN mitochondrial tRNA wobble uridine modification (ortholog); tRNA wobble uridine modification (ortholog); ASSOCIATED WITH combined oxidative phosphorylation deficiency (ortholog); combined oxidative p hosphorylation deficiency 10 (ortholog); Developmental Disabilities (ortholog); FOUND IN GTPBP3-MTO1 complex (ortholog) | | | | Naked Mole-Rat | 22 | description | gene, protein-coding, MODEL [RefSeq] |
| 14107937 | MTO1 | mitochondrial tRNA translation optimization 1 | ENCODES a protein that exhibits flavin adenine dinucleotide binding (inferred); tRNA 5-taurinomethyluridine synthase activity (inferred); INVOLVED IN mitochondrial tRNA wobble uridine modification (ortholog); tRNA wobble uridine modification (ortholog); ASSOCIATED WITH combined oxidative phosphoryla tion deficiency (ortholog); combined oxidative phosphorylation deficiency 10 (ortholog); Developmental Disabilities (ortholog); FOUND IN cytoplasm (inferred); GTPBP3-MTO1 complex (inferred); mitochondrion (inferred); INTERACTS WITH deoxynivalenol | | | | Pig | 23 | description | gene, protein-coding, MODEL [RefSeq] |
| 626003388 | Mto1 | mitochondrial tRNA translation optimization 1 | ENCODES a protein that exhibits tRNA 5-taurinomethyluridine synthase activity (ortholog); INVOLVED IN mitochondrial tRNA wobble uridine modification (ortholog); tRNA wobble uridine modification (ortholog); ASSOCIATED WITH combined oxidative phosphorylation deficiency (ortholog); combined oxidative p hosphorylation deficiency 10 (ortholog); Developmental Disabilities (ortholog); FOUND IN GTPBP3-MTO1 complex (ortholog); mitochondrion (ortholog) | | | | Black Rat | 14 | description | gene, protein-coding, MODEL [RefSeq] |