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MOUSE:FRDA

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Contents

Species (Taxon ID) Mus musculus (Mouse). ([1])
Gene Name(s) Fxn (synonyms: Frda)
Protein Name(s) Frataxin, mitochondrial

Fxn Frataxin intermediate form Frataxin mature form

External Links
EMBL U95736
IPI IPI00136201
RefSeq NP_032070.1
UniGene Mm.7319
ProteinModelPortal O35943
SMR O35943
STRING O35943
PRIDE O35943
Ensembl ENSMUST00000081333
GeneID 14297
KEGG mmu:14297
CTD 2395
MGI MGI:1096879
eggNOG roNOG14548
HOGENOM HBG125781
HOVERGEN HBG005745
InParanoid O35943
OMA AYSGKGT
OrthoDB EOG4ZCT5S
PhylomeDB O35943
NextBio 285709
ArrayExpress O35943
Bgee O35943
CleanEx MM_FXN
Genevestigator O35943
GermOnline ENSMUSG00000059363
GO GO:0005739
GO:0004322
GO:0007628
GO:0009060
GO:0006879
GO:0009792
GO:0006783
GO:0006811
GO:0007005
GO:0040015
GO:0046621
GO:0006119
GO:0019230
InterPro IPR017789
IPR002908
IPR020895
IPR001794
Gene3D G3DSA:3.30.920.10
PANTHER PTHR16821
Pfam PF01491
PRINTS PR00904
SUPFAM SSF55387
TIGRFAMs TIGR03421
TIGR03422
PROSITE PS01344
PS50810

Annotations

Qualifier GO ID GO term name Reference Evidence Code with/from Aspect Notes Status
GO:0004322

ferroxidase activity

GO_REF:0000002

IEA: Inferred from Electronic Annotation

InterPro:IPR001794

F

Seeded From UniProt

GO:0004322

ferroxidase activity

GO_REF:0000002

IEA: Inferred from Electronic Annotation

InterPro:IPR017789

F

Seeded From UniProt

GO:0004322

ferroxidase activity

GO_REF:0000003

IEA: Inferred from Electronic Annotation

EC:1.16.3.1

F

Seeded From UniProt

GO:0004322

ferroxidase activity

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

F

Seeded From UniProt

GO:0005737

cytoplasm

GO_REF:0000004

IEA: Inferred from Electronic Annotation

SP_KW:KW-0963

C

Seeded From UniProt

GO:0005737

cytoplasm

GO_REF:0000023

IEA: Inferred from Electronic Annotation

SP_SL:SL-0086

C

Seeded From UniProt

GO:0005739

mitochondrion

GO_REF:0000002

IEA: Inferred from Electronic Annotation

InterPro:IPR001794

C

Seeded From UniProt

GO:0005739

mitochondrion

GO_REF:0000004

IEA: Inferred from Electronic Annotation

SP_KW:KW-0496

C

Seeded From UniProt

GO:0005739

mitochondrion

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

C

Seeded From UniProt

GO:0005739

mitochondrion

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSRNOP00000020412

C

Seeded From UniProt

GO:0005739

mitochondrion

GO_REF:0000023

IEA: Inferred from Electronic Annotation

SP_SL:SL-0173

C

Seeded From UniProt

GO:0005739

mitochondrion

PMID:14651853[1]

IDA: Inferred from Direct Assay

C

Seeded From UniProt

GO:0005739

mitochondrion

PMID:17597094[2]

IDA: Inferred from Direct Assay

C

Seeded From UniProt

GO:0005739

mitochondrion

PMID:18614015[3]

IDA: Inferred from Direct Assay

C

Seeded From UniProt

GO:0005829

cytosol

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

C

Seeded From UniProt

GO:0006119

oxidative phosphorylation

PMID:16278235[4]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2176228

P

Seeded From UniProt

GO:0006119

oxidative phosphorylation

PMID:16278235[4]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177176

P

Seeded From UniProt

GO:0006119

oxidative phosphorylation

PMID:17404227[5]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177176

P

Seeded From UniProt

GO:0006119

oxidative phosphorylation

PMID:17404227[5]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2182103

P

Seeded From UniProt

GO:0006783

heme biosynthetic process

GO_REF:0000004

IEA: Inferred from Electronic Annotation

SP_KW:KW-0350

P

Seeded From UniProt

GO:0006810

transport

GO_REF:0000004

IEA: Inferred from Electronic Annotation

SP_KW:KW-0813

P

Seeded From UniProt

GO:0006811

ion transport

GO_REF:0000004

IEA: Inferred from Electronic Annotation

SP_KW:KW-0406

P

Seeded From UniProt

GO:0006879

cellular iron ion homeostasis

GO_REF:0000002

IEA: Inferred from Electronic Annotation

InterPro:IPR001794

P

Seeded From UniProt

GO:0006879

cellular iron ion homeostasis

GO_REF:0000004

IEA: Inferred from Electronic Annotation

SP_KW:KW-0409

P

Seeded From UniProt

GO:0006879

cellular iron ion homeostasis

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

P

Seeded From UniProt

GO:0006879

cellular iron ion homeostasis

PMID:9405681[6]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177175

P

Seeded From UniProt

GO:0006879

cellular iron ion homeostasis

PMID:9405681[6]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177176

P

Seeded From UniProt

GO:0007005

mitochondrion organization

PMID:14985441[7]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177177

P

Seeded From UniProt

GO:0007005

mitochondrion organization

PMID:9405681[6]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177175

P

Seeded From UniProt

GO:0007005

mitochondrion organization

PMID:9405681[6]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177176

P

Seeded From UniProt

GO:0007628

adult walking behavior

PMID:14985441[7]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177177

P

Seeded From UniProt

GO:0007628

adult walking behavior

PMID:9405681[6]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177175

P

Seeded From UniProt

GO:0007628

adult walking behavior

PMID:9405681[6]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177176

P

Seeded From UniProt

GO:0008198

ferrous iron binding

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

F

Seeded From UniProt

GO:0008199

ferric iron binding

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

F

Seeded From UniProt

GO:0008284

positive regulation of cell proliferation

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

P

Seeded From UniProt

GO:0009060

aerobic respiration

PMID:16278235[4]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2176228

P

Seeded From UniProt

GO:0009060

aerobic respiration

PMID:16278235[4]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177176

P

Seeded From UniProt

GO:0009060

aerobic respiration

PMID:9405681[6]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177175

P

Seeded From UniProt

GO:0009060

aerobic respiration

PMID:9405681[6]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177176

P

Seeded From UniProt

GO:0009792

embryo development ending in birth or egg hatching

PMID:10767347[8]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177162

P

Seeded From UniProt

GO:0010039

response to iron ion

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

P

Seeded From UniProt

GO:0010722

regulation of ferrochelatase activity

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

P

Seeded From UniProt

GO:0014070

response to organic cyclic compound

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSRNOP00000020412

P

Seeded From UniProt

GO:0016226

iron-sulfur cluster assembly

PMID:16278235[4]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2176228

P

Seeded From UniProt

GO:0016226

iron-sulfur cluster assembly

PMID:16278235[4]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177176

P

Seeded From UniProt

GO:0016226

iron-sulfur cluster assembly

PMID:17597094[2]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2176228

P

Seeded From UniProt

GO:0016226

iron-sulfur cluster assembly

PMID:17597094[2]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177176

P

Seeded From UniProt

GO:0016491

oxidoreductase activity

GO_REF:0000004

IEA: Inferred from Electronic Annotation

SP_KW:KW-0560

F

Seeded From UniProt

GO:0016540

protein autoprocessing

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

P

Seeded From UniProt

GO:0018283

iron incorporation into metallo-sulfur cluster

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

P

Seeded From UniProt

GO:0019230

proprioception

PMID:11175786[9]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177175

P

Seeded From UniProt

GO:0019230

proprioception

PMID:11175786[9]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177176

P

Seeded From UniProt

GO:0019230

proprioception

PMID:14985441[7]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177177

P

Seeded From UniProt

GO:0019899

enzyme binding

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSRNOP00000020412

F

Seeded From UniProt

GO:0030307

positive regulation of cell growth

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

P

Seeded From UniProt

GO:0034986

iron chaperone activity

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

F

Seeded From UniProt

GO:0040015

negative regulation of multicellular organism growth

PMID:17404227[5]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177176

P

Seeded From UniProt

GO:0040015

negative regulation of multicellular organism growth

PMID:17404227[5]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2182103

P

Seeded From UniProt

GO:0042493

response to drug

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSRNOP00000020412

P

Seeded From UniProt

GO:0043066

negative regulation of apoptosis

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

P

Seeded From UniProt

GO:0045773

positive regulation of axon extension

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSRNOP00000020412

P

Seeded From UniProt

GO:0046621

negative regulation of organ growth

PMID:9405681[6]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177175

P

Seeded From UniProt

GO:0046621

negative regulation of organ growth

PMID:9405681[6]

IMP: Inferred from Mutant Phenotype

MGI:MGI:2177176

P

Seeded From UniProt

GO:0046872

metal ion binding

GO_REF:0000004

IEA: Inferred from Electronic Annotation

SP_KW:KW-0479

F

Seeded From UniProt

GO:0048554

positive regulation of metalloenzyme activity

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

P

Seeded From UniProt

GO:0051347

positive regulation of transferase activity

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

P

Seeded From UniProt

GO:0051349

positive regulation of lyase activity

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

P

Seeded From UniProt

GO:0051353

positive regulation of oxidoreductase activity

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

P

Seeded From UniProt

GO:0051536

iron-sulfur cluster binding

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

F

Seeded From UniProt

GO:0051537

2 iron, 2 sulfur cluster binding

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

F

Seeded From UniProt

GO:0055072

iron ion homeostasis

GO_REF:0000004

IEA: Inferred from Electronic Annotation

SP_KW:KW-0410

P

Seeded From UniProt

GO:0055114

oxidation-reduction process

GO_REF:0000002

IEA: Inferred from Electronic Annotation

InterPro:IPR001794

P

Seeded From UniProt

GO:0055114

oxidation-reduction process

GO_REF:0000002

IEA: Inferred from Electronic Annotation

InterPro:IPR017789

P

Seeded From UniProt

GO:0055114

oxidation-reduction process

GO_REF:0000004

IEA: Inferred from Electronic Annotation

SP_KW:KW-0560

P

Seeded From UniProt

GO:0070301

cellular response to hydrogen peroxide

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

P

Seeded From UniProt

GO:0090201

negative regulation of release of cytochrome c from mitochondria

GO_REF:0000019

IEA: Inferred from Electronic Annotation

Ensembl:ENSP00000366482

P

Seeded From UniProt


Notes

References

See Help:References for how to manage references in GONUTS.

  1. Mootha VK et al. (2003) Integrated analysis of protein composition, tissue diversity, and gene regulation in mouse mitochondria. Cell 115: 629-40 PubMed GONUTS page
  2. 2.0 2.1 2.2 Martelli A et al. (2007) Frataxin is essential for extramitochondrial Fe-S cluster proteins in mammalian tissues. Hum Mol Genet 16: 2651-8 PubMed GONUTS page
  3. Pagliarini DJ et al. (2008) A mitochondrial protein compendium elucidates complex I disease biology. Cell 134: 112-23 PubMed GONUTS page
  4. 4.0 4.1 4.2 4.3 4.4 4.5 Thierbach R et al. (2005) Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in mice. Hum Mol Genet 14: 3857-64 PubMed GONUTS page
  5. 5.0 5.1 5.2 5.3 Pomplun D et al. (2007) Reduced expression of mitochondrial frataxin in mice exacerbates diet-induced obesity. Proc Natl Acad Sci U S A 104: 6377-81 PubMed GONUTS page
  6. 6.0 6.1 6.2 6.3 6.4 6.5 6.6 6.7 6.8 6.9 Oliver PM et al. (1997) Hypertension, cardiac hypertrophy, and sudden death in mice lacking natriuretic peptide receptor A. Proc Natl Acad Sci U S A 94: 14730-5 PubMed GONUTS page
  7. 7.0 7.1 7.2 Simon D et al. (2004) Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia. J Neurosci 24: 1987-95 PubMed GONUTS page
  8. Cossée M et al. (2000) Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation. Hum Mol Genet 9: 1219-26 PubMed GONUTS page
  9. 9.0 9.1 Puccio H et al. (2001) Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits. Nat Genet 27: 181-6 PubMed GONUTS page
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