Gene
mto1
- ID
- ZDB-GENE-070209-253
- Name
- mitochondrial tRNA translation optimization 1
- Symbol
- mto1 Nomenclature History
- Previous Names
-
- zgc:158782
- Type
- protein_coding_gene
- Location
- Chr: 12 Mapping Details/Browsers
- Description
- Predicted to enable flavin adenine dinucleotide binding activity. Predicted to be involved in mitochondrial tRNA wobble uridine modification and tRNA methylation. Predicted to act upstream of or within tRNA wobble uridine modification. Predicted to be located in cytoplasm. Predicted to be active in cytosol and mitochondrion. Is expressed in brain and heart. Human ortholog(s) of this gene implicated in combined oxidative phosphorylation deficiency 10. Orthologous to human MTO1 (mitochondrial tRNA translation optimization 1).
- Genome Resources
- Note
- None
- Comparative Information
-
- All Expression Data
- 3 figures from 2 publications
- Cross-Species Comparison
- High Throughput Data
- Thisse Expression Data
- No data available
Wild Type Expression Summary
- All Phenotype Data
- 8 figures from Zhang et al., 2021
- Cross-Species Comparison
- Alliance
Phenotype Summary
Mutations
Human Disease
| Disease Ontology Term | Multi-Species Data | OMIM Term | OMIM Phenotype ID |
|---|---|---|---|
| combined oxidative phosphorylation deficiency 10 | Alliance | Combined oxidative phosphorylation deficiency 10 | 614702 |
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Domain, Family, and Site Summary
| Type | InterPro ID | Name |
|---|---|---|
| Conserved_site | IPR020595 | MnmG-related, conserved site |
| Domain | IPR026904 | tRNA uridine 5-carboxymethylaminomethyl modification enzyme MnmG, C-terminal |
| Domain | IPR040131 | MnmG, N-terminal domain |
| Domain | IPR047001 | tRNA uridine 5-carboxymethylaminomethyl modification enzyme, C-terminal subdomain |
| Domain | IPR049312 | tRNA uridine 5-carboxymethylaminomethyl modification enzyme, C-terminal, N-terninal subdomain |
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Domain Details Per Protein
| Protein | Additional Resources | Length | FAD/NAD(P)-binding domain superfamily | MnmG, N-terminal domain | MnmG-related, conserved site | tRNA uridine 5-carboxymethylaminomethyl modification enzyme, C-terminal, N-terninal subdomain | tRNA uridine 5-carboxymethylaminomethyl modification enzyme, C-terminal subdomain | tRNA uridine 5-carboxymethylaminomethyl modification enzyme, C-terminal subdomain superfamily | tRNA uridine 5-carboxymethylaminomethyl modification enzyme MnmG | tRNA uridine 5-carboxymethylaminomethyl modification enzyme MnmG, C-terminal | tRNA uridine 5-carboxymethylaminomethyl modification enzyme MnmG-related |
|---|---|---|---|---|---|---|---|---|---|---|---|
| UniProtKB:A0A8M9QE21 | InterPro | 570 | |||||||||
| UniProtKB:A0A8M3B787 | InterPro | 695 | |||||||||
| UniProtKB:A2RV35 | InterPro | 659 | |||||||||
| UniProtKB:F1R509 | InterPro | 659 |
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Interactions and Pathways
No data available
Plasmids
No data available
No data available
| Relationship | Marker Type | Marker | Accession Numbers | Citations |
|---|---|---|---|---|
| Contained in | BAC | CH73-119O17 | ZFIN Curated Data | |
| Contained in | BAC | RP71-1H7 | ZFIN Curated Data | |
| Encodes | cDNA | MGC:158782 | ZFIN Curated Data |
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| Type | Accession # | Sequence | Length (nt/aa) | Analysis |
|---|---|---|---|---|
| RNA | RefSeq:NM_001083009 (1) | 2201 nt | ||
| Genomic | GenBank:AL627167 (2) | 156685 nt | ||
| Polypeptide | UniProtKB:A0A8M3B787 (1) | 695 aa |
- Zhang, Q., He, X., Yao, S., Lin, T., Zhang, L., Chen, D., Chen, C., Yang, Q., Li, F., Zhu, Y.M., Guan, M.X. (2021) Ablation of Mto1 in zebrafish exhibited hypertrophic cardiomyopathy manifested by mitochondrion RNA maturation deficiency. Nucleic acids research. 49(8):4689-4704
- Chen, D., Zhang, Z., Chen, C., Yao, S., Yang, Q., Li, F., He, X., Ai, C., Wang, M., Guan, M.X. (2019) Deletion of Gtpbp3 in zebrafish revealed the hypertrophic cardiomyopathy manifested by aberrant mitochondrial tRNA metabolism. Nucleic acids research. 47(10):5341-5355
- Cheung, C.T., Nguyen, T.V., Le Cam, A., Patinote, A., Journot, L., Reynes, C., Bobe, J. (2019) What makes a bad egg? Egg transcriptome reveals dysregulation of translational machinery and novel fertility genes important for fertilization. BMC Genomics. 20:584
- Elkon, R., Milon, B., Morrison, L., Shah, M., Vijayakumar, S., Racherla, M., Leitch, C.C., Silipino, L., Hadi, S., Weiss-Gayet, M., Barras, E., Schmid, C.D., Ait-Lounis, A., Barnes, A., Song, Y., Eisenman, D.J., Eliyahu, E., Frolenkov, G.I., Strome, S.E., Durand, B., Zaghloul, N.A., Jones, S.M., Reith, W., Hertzano, R. (2015) RFX transcription factors are essential for hearing in mice. Nature communications. 6:8549
- Strausberg,R.L., Feingold,E.A., Grouse,L.H., Derge,J.G., Klausner,R.D., Collins,F.S., Wagner,L., Shenmen,C.M., Schuler,G.D., Altschul,S.F., Zeeberg,B., Buetow,K.H., Schaefer,C.F., Bhat,N.K., Hopkins,R.F., Jordan,H., Moore,T., Max,S.I., Wang,J., Hsieh,F., Diatchenko,L., Marusina,K., Farmer,A.A., Rubin,G.M., Hong,L., Stapleton,M., Soares,M.B., Bonaldo,M.F., Casavant,T.L., Scheetz,T.E., Brownstein,M.J., Usdin,T.B., Toshiyuki,S., Carninci,P., Prange,C., Raha,S.S., Loquellano,N.A., Peters,G.J., Abramson,R.D., Mullahy,S.J., Bosak,S.A., McEwan,P.J., McKernan,K.J., Malek,J.A., Gunaratne,P.H., Richards,S., Worley,K.C., Hale,S., Garcia,A.M., Gay,L.J., Hulyk,S.W., Villalon,D.K., Muzny,D.M., Sodergren,E.J., Lu,X., Gibbs,R.A., Fahey,J., Helton,E., Ketteman,M., Madan,A., Rodrigues,S., Sanchez,A., Whiting,M., Madan,A., Young,A.C., Shevchenko,Y., Bouffard,G.G., Blakesley,R.W., Touchman,J.W., Green,E.D., Dickson,M.C., Rodriguez,A.C., Grimwood,J., Schmutz,J., Myers,R.M., Butterfield,Y.S., Krzywinski,M.I., Skalska,U., Smailus,D.E., Schnerch,A., Schein,J.E., Jones,S.J., and Marra,M.A. (2002) Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences. Proceedings of the National Academy of Sciences of the United States of America. 99(26):16899-903
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