mtcu-2 Protein MTO1 homolog, mitochondrial [Caenorhabditis elegans] - Gene

Summary

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Official Symbol: mtcu-2
Official Full Name: Protein MTO1 homolog, mitochondrial
Primary source: WormBase:WBGene00009944
Locus tag: CELE_F52H3.2
See related: AllianceGenome:WB:WBGene00009944
Gene type: protein coding
RefSeq status: REVIEWED
Organism: Caenorhabditis elegans (strain: Bristol N2)
Lineage: Eukaryota; Metazoa; Ecdysozoa; Nematoda; Chromadorea; Rhabditida; Rhabditina; Rhabditomorpha; Rhabditoidea; Rhabditidae; Peloderinae; Caenorhabditis
Summary: Predicted to enable flavin adenine dinucleotide binding activity. Predicted to be involved in mitochondrial tRNA wobble uridine modification and tRNA methylation. Predicted to be active in cytosol and mitochondrion. Human ortholog(s) of this gene implicated in combined oxidative phosphorylation deficiency 10. Orthologous to human MTO1 (mitochondrial tRNA translation optimization 1). [provided by Alliance of Genome Resources, Nov 2024]
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Genomic context

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See mtcu-2 in Genome Data Viewer

Location:: chromosome: II

Exon count:: 6

Sequence:: Chromosome: II; NC_003280.10 (10030889..10033213, complement)

Chromosome II - NC_003280.10 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:: NC_003280.10

Go to nucleotide: Graphics FASTA GenBank

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

We further show that mtcu-1; mttu-1 and mtcu-2; mttu-1 double mutants display severe growth defects and sterility. The animal models presented here support the idea that the pathological states in humans may initially develop not as a direct consequence of a bioenergetic defect, but from the cell's maladaptive response to the hypomodification status of mt-tRNAs
Title: Mutations in the Caenorhabditis elegans orthologs of human genes required for mitochondrial tRNA modification cause similar electron transport chain defects but different nuclear responses.

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Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

F52H3.2 (e-PCR)
- UniSTS:307684

Homology

Orthologs from OrthoDB

Gene Ontology Provided by WormBase

Function	Evidence Code	Pubs
enables flavin adenine dinucleotide binding	IBA Inferred from Biological aspect of Ancestor more info
enables flavin adenine dinucleotide binding	IEA Inferred from Electronic Annotation more info

Process	Evidence Code	Pubs
involved_in mitochondrial tRNA wobble uridine modification	IBA Inferred from Biological aspect of Ancestor more info
involved_in tRNA methylation	IBA Inferred from Biological aspect of Ancestor more info
involved_in tRNA processing	IEA Inferred from Electronic Annotation more info
involved_in tRNA wobble uridine modification	IEA Inferred from Electronic Annotation more info

Component	Evidence Code	Pubs
is_active_in cytosol	IBA Inferred from Biological aspect of Ancestor more info
is_active_in mitochondrion	IBA Inferred from Biological aspect of Ancestor more info

General protein information

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Preferred Names: Protein MTO1 homolog, mitochondrial

NP_496169.1

Confirmed by transcript evidence

NCBI Reference Sequences (RefSeq)

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Genome Annotation

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference assembly

Genomic

NC_003280.10 Reference assembly

Range

10030889..10033213 complement

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GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

NM_063768.8 → NP_496169.1 Protein MTO1 homolog, mitochondrial [Caenorhabditis elegans]

See identical proteins and their annotated locations for NP_496169.1

Status: REVIEWED

UniProtKB/Swiss-Prot

Q20680

Conserved Domains (3) summary

PRK05192
Location:18 → 629

PRK05192; tRNA uridine 5-carboxymethylaminomethyl modification enzyme GidA; Validated

pfam13932
Location:413 → 621

GIDA_assoc; GidA associated domain

cl21454
Location:330 → 408

NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins