FRDA_BOVIN
ID FRDA_BOVIN Reviewed; 217 AA.
AC Q05B87;
DT 29-MAY-2007, integrated into UniProtKB/Swiss-Prot.
DT 14-NOV-2006, sequence version 1.
DT 03-AUG-2022, entry version 90.
DE RecName: Full=Frataxin, mitochondrial;
DE Short=Fxn;
DE EC=1.16.3.1;
DE Contains:
DE RecName: Full=Frataxin intermediate form;
DE Contains:
DE RecName: Full=Frataxin mature form;
DE Flags: Precursor;
GN Name=FXN; Synonyms=FRDA;
OS Bos taurus (Bovine).
OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia;
OC Eutheria; Laurasiatheria; Artiodactyla; Ruminantia; Pecora; Bovidae;
OC Bovinae; Bos.
OX NCBI_TaxID=9913;
RN [1]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RC STRAIN=Hereford; TISSUE=Thalamus;
RG NIH - Mammalian Gene Collection (MGC) project;
RL Submitted (AUG-2006) to the EMBL/GenBank/DDBJ databases.
CC -!- FUNCTION: Promotes the biosynthesis of heme and assembly and repair of
CC iron-sulfur clusters by delivering Fe(2+) to proteins involved in these
CC pathways. May play a role in the protection against iron-catalyzed
CC oxidative stress through its ability to catalyze the oxidation of
CC Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in
CC vitro ferroxidase activity. May be able to store large amounts of iron
CC in the form of a ferrihydrite mineral by oligomerization. Modulates the
CC RNA-binding activity of ACO1 (By similarity). {ECO:0000250}.
CC -!- CATALYTIC ACTIVITY:
CC Reaction=4 Fe(2+) + 4 H(+) + O2 = 4 Fe(3+) + 2 H2O;
CC Xref=Rhea:RHEA:11148, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378,
CC ChEBI:CHEBI:15379, ChEBI:CHEBI:29033, ChEBI:CHEBI:29034; EC=1.16.3.1;
CC -!- SUBUNIT: Monomer (probable predominant form). Oligomer. Interacts with
CC LYRM4 and HSPA9. Interacts with ACO1. Interacts with ISCU. Interacts
CC with FECH; one iron-bound FXN monomer seems to interact with a FECH
CC homodimer. Interacts with SDHA and SDHB. Interacts with ACO2; the
CC interaction is dependent on citrate. {ECO:0000250|UniProtKB:D3ZYW7,
CC ECO:0000250|UniProtKB:Q16595}.
CC -!- SUBCELLULAR LOCATION: Cytoplasm {ECO:0000250|UniProtKB:Q16595}.
CC Mitochondrion {ECO:0000250|UniProtKB:Q16595}.
CC -!- PTM: Processed in two steps by mitochondrial processing peptidase
CC (MPP). MPP first cleaves the precursor to intermediate form and
CC subsequently converts the intermediate to yield frataxin mature form
CC (By similarity). {ECO:0000250}.
CC -!- SIMILARITY: Belongs to the frataxin family. {ECO:0000305}.
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DR EMBL; BC122620; AAI22621.1; -; mRNA.
DR RefSeq; NP_001074196.1; NM_001080727.1.
DR AlphaFoldDB; Q05B87; -.
DR SMR; Q05B87; -.
DR STRING; 9913.ENSBTAP00000001725; -.
DR PaxDb; Q05B87; -.
DR PRIDE; Q05B87; -.
DR Ensembl; ENSBTAT00000001725; ENSBTAP00000001725; ENSBTAG00000001306.
DR GeneID; 505694; -.
DR KEGG; bta:505694; -.
DR CTD; 2395; -.
DR VEuPathDB; HostDB:ENSBTAG00000001306; -.
DR VGNC; VGNC:29155; FXN.
DR eggNOG; KOG3413; Eukaryota.
DR GeneTree; ENSGT00390000005811; -.
DR HOGENOM; CLU_080880_1_0_1; -.
DR InParanoid; Q05B87; -.
DR OMA; CWINLRT; -.
DR OrthoDB; 1372185at2759; -.
DR TreeFam; TF318958; -.
DR Reactome; R-BTA-1362409; Mitochondrial iron-sulfur cluster biogenesis.
DR Proteomes; UP000009136; Chromosome 8.
DR Bgee; ENSBTAG00000001306; Expressed in supraspinatus muscle and 105 other tissues.
DR GO; GO:1990221; C:L-cysteine desulfurase complex; IEA:Ensembl.
DR GO; GO:0005739; C:mitochondrion; IBA:GO_Central.
DR GO; GO:0051537; F:2 iron, 2 sulfur cluster binding; IBA:GO_Central.
DR GO; GO:0008199; F:ferric iron binding; IBA:GO_Central.
DR GO; GO:0008198; F:ferrous iron binding; IBA:GO_Central.
DR GO; GO:0004322; F:ferroxidase activity; IBA:GO_Central.
DR GO; GO:0034986; F:iron chaperone activity; IBA:GO_Central.
DR GO; GO:0007628; P:adult walking behavior; IEA:Ensembl.
DR GO; GO:0006879; P:cellular iron ion homeostasis; IBA:GO_Central.
DR GO; GO:0070301; P:cellular response to hydrogen peroxide; IEA:Ensembl.
DR GO; GO:0009792; P:embryo development ending in birth or egg hatching; IEA:Ensembl.
DR GO; GO:0006783; P:heme biosynthetic process; IEA:UniProtKB-KW.
DR GO; GO:0006811; P:ion transport; IEA:UniProtKB-KW.
DR GO; GO:0018283; P:iron incorporation into metallo-sulfur cluster; IBA:GO_Central.
DR GO; GO:0007005; P:mitochondrion organization; IEA:Ensembl.
DR GO; GO:0046716; P:muscle cell cellular homeostasis; IEA:Ensembl.
DR GO; GO:0040015; P:negative regulation of multicellular organism growth; IEA:Ensembl.
DR GO; GO:0046621; P:negative regulation of organ growth; IEA:Ensembl.
DR GO; GO:0090201; P:negative regulation of release of cytochrome c from mitochondria; IEA:Ensembl.
DR GO; GO:0035265; P:organ growth; IEA:Ensembl.
DR GO; GO:0006119; P:oxidative phosphorylation; IEA:Ensembl.
DR GO; GO:1904234; P:positive regulation of aconitate hydratase activity; IEA:Ensembl.
DR GO; GO:0030307; P:positive regulation of cell growth; IEA:Ensembl.
DR GO; GO:0008284; P:positive regulation of cell population proliferation; IEA:Ensembl.
DR GO; GO:1904231; P:positive regulation of succinate dehydrogenase activity; IEA:Ensembl.
DR GO; GO:0019230; P:proprioception; IEA:Ensembl.
DR GO; GO:0016540; P:protein autoprocessing; IEA:Ensembl.
DR GO; GO:0010722; P:regulation of ferrochelatase activity; IEA:Ensembl.
DR GO; GO:0010039; P:response to iron ion; IEA:Ensembl.
DR Gene3D; 3.30.920.10; -; 1.
DR InterPro; IPR017789; Frataxin.
DR InterPro; IPR002908; Frataxin/CyaY.
DR InterPro; IPR036524; Frataxin/CyaY_sf.
DR InterPro; IPR020895; Frataxin_CS.
DR PANTHER; PTHR16821; PTHR16821; 1.
DR Pfam; PF01491; Frataxin_Cyay; 1.
DR PRINTS; PR00904; FRATAXIN.
DR SMART; SM01219; Frataxin_Cyay; 1.
DR SUPFAM; SSF55387; SSF55387; 1.
DR TIGRFAMs; TIGR03421; FeS_CyaY; 1.
DR TIGRFAMs; TIGR03422; mito_frataxin; 1.
DR PROSITE; PS01344; FRATAXIN_1; 1.
DR PROSITE; PS50810; FRATAXIN_2; 1.
PE 2: Evidence at transcript level;
KW Cytoplasm; Heme biosynthesis; Ion transport; Iron; Iron storage;
KW Iron transport; Mitochondrion; Oxidoreductase; Reference proteome;
KW Transit peptide; Transport.
FT TRANSIT 1..42
FT /note="Mitochondrion"
FT /evidence="ECO:0000250"
FT CHAIN 43..217
FT /note="Frataxin intermediate form"
FT /id="PRO_0000289330"
FT CHAIN 82..217
FT /note="Frataxin mature form"
FT /evidence="ECO:0000250"
FT /id="PRO_0000399387"
SQ SEQUENCE 217 AA; 23567 MW; AF8AB00650CD9ACC CRC64;
MWTLGRRSVA SFLPRSALPG FAPTRAGAPR PAKDLSLSGL PGLRIGTAKA PARSQSSLSL
RCLNQTLDVK KQSVCWINLR TAGTLGDAGT LDDTTYERLA EETLDSLAEF FEDLADKPYT
FEDYDVSFGS GVLTVKLGGD LGTYVINKQT PNKQIWLSSP SSGPKRYDWT GRNWVYSHDG
VSLHELLATE LTQALKTKLD LSALAYSGKD TCCPAQC
