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- Table of Contents
Facts about Protein MTO1 homolog, mitochondrial.
| Human | |
|---|---|
| Gene Name: | MTO1 |
| Uniprot: | Q9Y2Z2 |
| Entrez: | 25821 |

| Belongs to: |
|---|
| MnmG family |

EC 1.1.1.40; EC 6.3.5; homolog of yeast Mto1; mitochondrial MTO1-3; mitochondrial translation optimization 1 homolog (S. cerevisiae); protein MTO1 homolog, mitochondrial
Mass (kDA):
79.964 kDA

| Human | |
|---|---|
| Location: | 6q13 |
| Sequence: | 6; NC_000006.12 (73461737..73509236) |
Ubiquitously expressed in various tissues, but with a markedly elevated expression in tissues of high metabolic rates including cochlea.
Mitochondrion.




PMID: 12011058 by Li X.M., et al. Isolation and characterization of the putative nuclear modifier gene MTO1 involved in the pathogenesis of deafness-associated mitochondrial 12 S rRNA A1555G mutation.
PMID: 22608499 by Ghezzi D., et al. Mutations of the mitochondrial-tRNA modifier MTO1 cause hypertrophic cardiomyopathy and lactic acidosis.