InnateDB: Systems Biology of the Innate Immune Response

Mus musculus Protein: Fxn

Summary

InnateDB Protein

IDBP-154570.6

Last Modified

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

Gene Symbol

Fxn

Protein Name

frataxin

Synonyms

FA; FARR; Frda; X25;

Species

Mus musculus

Ensembl Protein

ENSMUSP00000080081

InnateDB Gene

IDBG-154568 (Fxn)

Protein Structure

UniProt Annotation

Function

Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization. Modulates the RNA-binding activity of ACO1 (By similarity). {ECO:0000250}.

Subcellular Localization

Cytoplasm {ECO:0000250}. Mitochondrion {ECO:0000269PubMed:17597094, ECO:0000269PubMed:9241270}. Note=PubMed:17597094 describes localization exclusively in mitochondria.

Disease Associations

Tissue Specificity

Heart, liver, skeletal muscle, kidney, spleen and thymus. Weakly expressed in the brain and lung. {ECO:0000269PubMed:9241270}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 5 experimentally validated interaction(s) in this database.
They are also associated with 6 interaction(s) predicted by orthology.

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

Predicted by orthology
Total	6 [view]

Gene Ontology

Molecular Function

Accession	GO Term
GO:0004322	ferroxidase activity
GO:0005515	protein binding
GO:0008198	ferrous iron binding
GO:0008199	ferric iron binding
GO:0034986	iron chaperone activity
GO:0051536	iron-sulfur cluster binding
GO:0051537	2 iron, 2 sulfur cluster binding

Biological Process

GO:0006119	oxidative phosphorylation
GO:0006783	heme biosynthetic process
GO:0006811	ion transport
GO:0006879	cellular iron ion homeostasis
GO:0007005	mitochondrion organization
GO:0007628	adult walking behavior
GO:0008284	positive regulation of cell proliferation
GO:0009060	aerobic respiration
GO:0009792	embryo development ending in birth or egg hatching
GO:0010039	response to iron ion
GO:0010722	regulation of ferrochelatase activity
GO:0016226	iron-sulfur cluster assembly
GO:0016540	protein autoprocessing
GO:0018283	iron incorporation into metallo-sulfur cluster
GO:0019230	proprioception
GO:0030307	positive regulation of cell growth
GO:0040015	negative regulation of multicellular organism growth
GO:0043066	negative regulation of apoptotic process
GO:0046621	negative regulation of organ growth
GO:0048554	positive regulation of metalloenzyme activity
GO:0051347	positive regulation of transferase activity
GO:0051349	positive regulation of lyase activity
GO:0051353	positive regulation of oxidoreductase activity
GO:0055114	oxidation-reduction process
GO:0070301	cellular response to hydrogen peroxide
GO:0090201	negative regulation of release of cytochrome c from mitochondria