frh-1 Frataxin, mitochondrial [Caenorhabditis elegans] - Gene

Summary

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Official Symbol: frh-1
Official Full Name: Frataxin, mitochondrial
Primary source: WormBase:WBGene00001486
Locus tag: CELE_F59G1.7
See related: AllianceGenome:WB:WBGene00001486
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 several functions, including 2 iron, 2 sulfur cluster binding activity; ferroxidase activity; and iron ion binding activity. Involved in determination of adult lifespan; response to hydrogen peroxide; and response to superoxide. Located in mitochondrion. Is expressed in amphid neurons; body wall musculature; intestine; pharyngeal muscle cell; and spermatheca. Used to study Friedreich ataxia. Human ortholog(s) of this gene implicated in Friedreich ataxia; Friedreich ataxia 1; glioblastoma; and type 2 diabetes mellitus. Orthologous to human FXN (frataxin). [provided by Alliance of Genome Resources, Dec 2024]
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Genomic context

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See frh-1 in Genome Data Viewer

Location:: chromosome: II

Exon count:: 3

Sequence:: Chromosome: II; NC_003280.10 (5917422..5918564)

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?

Demonstrate a causal connection between induction of autophagy and lifespan extension following reduced frataxin expression.
Title: Autophagy induction extends lifespan and reduces lipid content in response to frataxin silencing in C. elegans.
Frataxin suppression by RNAi significantly reduces lifespan in C. elegans.
Title: Impaired respiration is positively correlated with decreased life span in Caenorhabditis elegans models of Friedreich Ataxia.
Study describes in detail the structure of all of the coding units within the CEOP2232 operon, coding for frataxin; it is composed of eight genes of a diverse nature, organized in a complex structure.
Title: The frataxin-encoding operon of Caenorhabditis elegans shows complex structure and regulation.
The genomic structure of frh-1, the development of an RNA interference model, and the interaction of frh-1 with mev-1 are reported.
Title: Reduction of Caenorhabditis elegans frataxin increases sensitivity to oxidative stress, reduces lifespan, and causes lethality in a mitochondrial complex II mutant.
Frataxin suppression by RNAi significantly extends lifespan in C. elegans.
Title: Reduced expression of frataxin extends the lifespan of Caenorhabditis elegans.

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Pathways from PubChem

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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

F59G1.7 (e-PCR)
- UniSTS:316879

Homology

Orthologs from OrthoDB

Gene Ontology Provided by WormBase

Function	Evidence Code	Pubs
enables 2 iron, 2 sulfur cluster binding	IBA Inferred from Biological aspect of Ancestor more info
enables ferric iron binding	IBA Inferred from Biological aspect of Ancestor more info
enables ferric iron binding	IEA Inferred from Electronic Annotation more info
enables ferrous iron binding	IBA Inferred from Biological aspect of Ancestor more info
enables ferroxidase activity	IBA Inferred from Biological aspect of Ancestor more info
enables ferroxidase activity	IEA Inferred from Electronic Annotation more info
enables iron chaperone activity	IBA Inferred from Biological aspect of Ancestor more info
enables oxidoreductase activity	IEA Inferred from Electronic Annotation more info

Process	Evidence Code	Pubs
involved_in determination of adult lifespan	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in heme biosynthetic process	IEA Inferred from Electronic Annotation more info
involved_in intracellular iron ion homeostasis	IEA Inferred from Electronic Annotation more info
involved_in iron incorporation into metallo-sulfur cluster	IEA Inferred from Electronic Annotation more info
involved_in iron ion transport	IEA Inferred from Electronic Annotation more info
involved_in iron-sulfur cluster assembly	IBA Inferred from Biological aspect of Ancestor more info
involved_in iron-sulfur cluster assembly	IEA Inferred from Electronic Annotation more info
involved_in iron-sulfur cluster assembly	ISS Inferred from Sequence or Structural Similarity more info	PubMed
involved_in response to hydrogen peroxide	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in response to superoxide	IMP Inferred from Mutant Phenotype more info	PubMed

Component	Evidence Code	Pubs
is_active_in mitochondrion	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrion	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrion	IEA Inferred from Electronic Annotation more info
located_in mitochondrion	ISS Inferred from Sequence or Structural Similarity more info	PubMed

General protein information

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Preferred Names: Frataxin, mitochondrial

NP_495183.1

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

5917422..5918564

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GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

NM_062782.4 → NP_495183.1 Frataxin, mitochondrial [Caenorhabditis elegans]

See identical proteins and their annotated locations for NP_495183.1

Status: REVIEWED

UniProtKB/Swiss-Prot

Q9TY03

Conserved Domains (1) summary

TIGR03422
Location:24 → 123

mito_frataxin; frataxin