Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Adrenodoxin, mitochondrial

Gene

FDX1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Essential for the synthesis of various steroid hormones (PubMed:20547883, PubMed:21636783). Participates in the reduction of mitochondrial cytochrome P450 for steroidogenesis (PubMed:20547883, PubMed:21636783). Transfers electrons from adrenodoxin reductase to CYP11A1, a cytochrome P450 that catalyzes cholesterol side-chain cleavage (PubMed:20547883, PubMed:21636783). Does not form a ternary complex with adrenodoxin reductase and CYP11A1 but shuttles between the two enzymes to transfer electrons (By similarity).By similarity2 Publications

Cofactori

[2Fe-2S] cluster2 PublicationsNote: Binds 1 [2Fe-2S] cluster.2 Publications

Redox potential

E0 is -448 mV.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi106Iron-sulfur (2Fe-2S)1
Metal bindingi112Iron-sulfur (2Fe-2S)1
Metal bindingi115Iron-sulfur (2Fe-2S)1
Metal bindingi152Iron-sulfur (2Fe-2S)1

GO - Molecular functioni

  • 2 iron, 2 sulfur cluster binding Source: UniProtKB
  • electron transfer activity Source: UniProtKB
  • iron ion binding Source: ProtInc

GO - Biological processi

Keywordsi

Biological processCholesterol metabolism, Electron transport, Lipid metabolism, Steroid metabolism, Steroidogenesis, Sterol metabolism, Transport
Ligand2Fe-2S, Iron, Iron-sulfur, Metal-binding

Enzyme and pathway databases

ReactomeiR-HSA-1362409 Mitochondrial iron-sulfur cluster biogenesis
R-HSA-196108 Pregnenolone biosynthesis
R-HSA-211976 Endogenous sterols
R-HSA-2395516 Electron transport from NADPH to Ferredoxin
R-HSA-5579026 Defective CYP11A1 causes Adrenal insufficiency, congenital, with 46,XY sex reversal (AICSR)

Names & Taxonomyi

Protein namesi
Recommended name:
Adrenodoxin, mitochondrial
Alternative name(s):
Adrenal ferredoxin
Ferredoxin-1
Hepatoredoxin
Gene namesi
Name:FDX1
Synonyms:ADX
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 11

Organism-specific databases

EuPathDBiHostDB:ENSG00000137714.2
HGNCiHGNC:3638 FDX1
MIMi103260 gene
neXtProtiNX_P10109

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

DisGeNETi2230
OpenTargetsiENSG00000137714
PharmGKBiPA28082

Chemistry databases

DrugBankiDB00648 Mitotane

Polymorphism and mutation databases

BioMutaiFDX1
DMDMi113471

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 60MitochondrionAdd BLAST60
ChainiPRO_000000098861 – 184Adrenodoxin, mitochondrialAdd BLAST124

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei63PhosphoserineBy similarity1
Modified residuei66N6-acetyllysine; alternateBy similarity1
Modified residuei66N6-succinyllysine; alternateBy similarity1
Modified residuei158N6-succinyllysineBy similarity1
Modified residuei177PhosphoserineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiP10109
MaxQBiP10109
PaxDbiP10109
PeptideAtlasiP10109
PRIDEiP10109
ProteomicsDBi52566
TopDownProteomicsiP10109

PTM databases

iPTMnetiP10109
PhosphoSitePlusiP10109

Expressioni

Tissue specificityi

Highest levels in the adrenal gland (at protein level). Also detected in kidney and testis (at protein level).1 Publication

Gene expression databases

BgeeiENSG00000137714 Expressed in 208 organ(s), highest expression level in metanephros
CleanExiHS_FDX1
GenevisibleiP10109 HS

Organism-specific databases

HPAiHPA041630
HPA062087

Interactioni

Subunit structurei

Interacts with CYP11A1.2 Publications

Protein-protein interaction databases

BioGridi108521, 11 interactors
IntActiP10109, 10 interactors
STRINGi9606.ENSP00000260270

Structurei

Secondary structure

1184
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliP10109
SMRiP10109
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP10109

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini67 – 1712Fe-2S ferredoxin-typePROSITE-ProRule annotationAdd BLAST105

Sequence similaritiesi

Belongs to the adrenodoxin/putidaredoxin family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG3309 Eukaryota
COG0633 LUCA
GeneTreeiENSGT00530000063577
HOGENOMiHOG000244518
HOVERGENiHBG000092
InParanoidiP10109
KOiK22070
OMAiDGLKVKM
OrthoDBiEOG091G0MJ2
PhylomeDBiP10109
TreeFamiTF319845

Family and domain databases

CDDicd00207 fer2, 1 hit
Gene3Di3.10.20.30, 1 hit
InterProiView protein in InterPro
IPR036010 2Fe-2S_ferredoxin-like_sf
IPR001041 2Fe-2S_ferredoxin-type
IPR001055 Adrenodoxin
IPR018298 Adrenodoxin_Fe-S_BS
IPR012675 Beta-grasp_dom_sf
PfamiView protein in Pfam
PF00111 Fer2, 1 hit
PRINTSiPR00355 ADRENODOXIN
SUPFAMiSSF54292 SSF54292, 1 hit
PROSITEiView protein in PROSITE
PS51085 2FE2S_FER_2, 1 hit
PS00814 ADX, 1 hit

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

P10109-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MAAAGGARLL RAASAVLGGP AGRWLHHAGS RAGSSGLLRN RGPGGSAEAS
60 70 80 90 100
RSLSVSARAR SSSEDKITVH FINRDGETLT TKGKVGDSLL DVVVENNLDI
110 120 130 140 150
DGFGACEGTL ACSTCHLIFE DHIYEKLDAI TDEENDMLDL AYGLTDRSRL
160 170 180
GCQICLTKSM DNMTVRVPET VADARQSIDV GKTS
Length:184
Mass (Da):19,393
Last modified:July 1, 1989 - v1
Checksum:iA234EC601136C85F
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M23668 Genomic DNA Translation: AAA50462.1
J03548 mRNA Translation: AAA96806.1
M18003 mRNA Translation: AAA76853.1
M34788 mRNA Translation: AAA35829.1
M34786, M34784 Genomic DNA Translation: AAA35856.1
M34785, M34783 Genomic DNA Translation: AAA35855.1
BT006681 mRNA Translation: AAP35327.1
EF444978 Genomic DNA Translation: ACA05992.1
BC010284 mRNA Translation: AAH10284.1
BC017063 mRNA Translation: AAH17063.1
CCDSiCCDS8344.1
PIRiA31853 AXHU
RefSeqiNP_004100.1, NM_004109.4
UniGeneiHs.744

Genome annotation databases

EnsembliENST00000260270; ENSP00000260270; ENSG00000137714
GeneIDi2230
KEGGihsa:2230
UCSCiuc001pkx.4 human

Similar proteinsi

Entry informationi

Entry nameiADX_HUMAN
AccessioniPrimary (citable) accession number: P10109
Secondary accession number(s): B0YJ14, Q53YD6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 1, 1989
Last sequence update: July 1, 1989
Last modified: September 12, 2018
This is version 182 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.

Do not show this banner again