InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: MTO1

Summary

InnateDB Protein

IDBP-383263.4

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

MTO1

Protein Name

mitochondrial translation optimization 1 homolog (S. cerevisiae)

Synonyms

COXPD10;

Species

Homo sapiens

Ensembl Protein

ENSP00000402038

InnateDB Gene

IDBG-92375 (MTO1)

Protein Structure

UniProt Annotation

Function

Involved in the 5-carboxymethylaminomethyl modification (mnm(5)s(2)U34) of the wobble uridine base in mitochondrial tRNAs. {ECO:0000269PubMed:12011058}.

Subcellular Localization

Mitochondrion {ECO:0000250}.

Disease Associations

Combined oxidative phosphorylation deficiency 10 (COXPD10) [MIM:614702]: An autosomal recessive disorder resulting in variable defects of mitochondrial oxidative respiration. Affected individuals present in infancy with hypertrophic cardiomyopathy and lactic acidosis. The severity is variable, but can be fatal in the most severe cases. {ECO:0000269PubMed:22608499}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Ubiquitously expressed in various tissues, but with a markedly elevated expression in tissues of high metabolic rates including cochlea. {ECO:0000269PubMed:12011058}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 5 experimentally validated interaction(s) in this database.

Experimentally validated
Total	5 [view]
Protein-Protein	5 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0044822	poly(A) RNA binding
GO:0050660	flavin adenine dinucleotide binding

Biological Process

GO:0002098	tRNA wobble uridine modification
GO:0008033	tRNA processing
GO:0055114	oxidation-reduction process
GO:0070899	mitochondrial tRNA wobble uridine modification

Cellular Component

GO:0005739

mitochondrion

Protein Structure and Domains

PDB ID

InterPro

IPR002218 Glucose-inhibited division protein A-related
IPR003953 FAD binding domain
IPR004416 Glucose-inhibited division protein A
IPR013027 FAD-dependent pyridine nucleotide-disulphide oxidoreductase

PFAM

PF01134
PF00890

PRINTS

PR00368

PIRSF

SMART

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

Q9Y2Z2