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Q99424 (ACOX2_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified April 16, 2014. Version 129. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (3) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Peroxisomal acyl-coenzyme A oxidase 2

EC=1.17.99.3
Alternative name(s):
3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanoyl-CoA 24-hydroxylase
3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanoyl-CoA oxidase
Trihydroxycoprostanoyl-CoA oxidase
Short name=THCA-CoA oxidase
Short name=THCCox
Gene names
Name:ACOX2
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length681 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

Oxidizes the CoA esters of the bile acid intermediates di- and tri-hydroxycholestanoic acids.

Catalytic activity

(25R)-3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestan-26-oyl-CoA + H2O + acceptor = (24R,25R)-3-alpha,7-alpha,12-alpha,24-tetrahydroxy-5-beta-cholestan-26-oyl-CoA + reduced acceptor.

Cofactor

FAD By similarity.

Subunit structure

Heterodimer By similarity.

Subcellular location

Peroxisome.

Tissue specificity

Present in all tissues tested: heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas. Most abundant in heart, liver and kidney.

Sequence similarities

Belongs to the acyl-CoA oxidase family.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 681681Peroxisomal acyl-coenzyme A oxidase 2
PRO_0000204681

Regions

Motif679 – 6813Microbody targeting signal Potential

Amino acid modifications

Modified residue31Phosphoserine By similarity
Modified residue661N6-succinyllysine By similarity
Modified residue1371N6-succinyllysine By similarity
Modified residue4531N6-succinyllysine By similarity
Modified residue5611N6-succinyllysine By similarity

Sequences

Sequence LengthMass (Da)Tools
Q99424 [UniParc].

Last modified May 1, 1997. Version 1.
Checksum: 7FAE9236FCBE6D4D

FASTA68176,827
        10         20         30         40         50         60 
MGSPVHRVSL GDTWSRQMHP DIESERYMQS FDVERLTNIL DGGAQNTALR RKVESIIHSY 

        70         80         90        100        110        120 
PEFSCKDNYF MTQNERYKAA MRRAFHIRLI ARRLGWLEDG RELGYAYRAL SGDVALNIHR 

       130        140        150        160        170        180 
VFVRALRSLG SEEQIAKWDP LCKNIQIIAT YAQTELGHGT YLQGLETEAT YDAATQEFVI 

       190        200        210        220        230        240 
HSPTLTATKW WPGDLGRSAT HALVQAQLIC SGARRGMHAF IVPIRSLQDH TPLPGIIIGD 

       250        260        270        280        290        300 
IGPKMDFDQT DNGFLQLNHV RVPRENMLSR FAQVLPDGTY VKLGTAQSNY LPMVVVRVEL 

       310        320        330        340        350        360 
LSGEILPILQ KACVIAMRYS VIRRQSRLRP SDPEAKVLDY QTQQQKLFPQ LAISYAFHFL 

       370        380        390        400        410        420 
AVSLLEFFQH SYTAILNQDF SFLPELHALS TGMKAMMSEF CTQGAEMCRR ACGGHGYSKL 

       430        440        450        460        470        480 
SGLPSLVTKL SASCTYEGEN TVLYLQVARF LVKSYLQTQM SPGSTPQRSL SPSVAYLTAP 

       490        500        510        520        530        540 
DLARCPAQRA ADFLCPELYT TAWAHVAVRL IKDSVQHLQT LTQSGADQHE AWNQTTVIHL 

       550        560        570        580        590        600 
QAAKVHCYYV TVKGFTEALE KLENEPAIQQ VLKRLCDLHA IHGILTNSGD FLHDAFLSGA 

       610        620        630        640        650        660 
QVDMARTAYL DLLRLIRKDA ILLTDAFDFT DQCLNSALGC YDGNVYERLF QWAQKSPTNT 

       670        680 
QENPAYEEYI RPLLQSWRSK L 

« Hide

References

« Hide 'large scale' references
[1]"Molecular characterization of the human peroxisomal branched-chain acyl-CoA oxidase: cDNA cloning, chromosomal assignment, tissue distribution, and evidence for the absence of the protein in Zellweger's syndrome."
Baumgart E., Vanhooren J.C.T., Fransen M., Marynen P., Puype M., Vandekerckhove J., Leunissen J.A.M., Fahimi H.D., Mannaerts G.P., Van Veldhoven P.P.
Proc. Natl. Acad. Sci. U.S.A. 93:13748-13753(1996) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA], PROTEIN SEQUENCE OF 8-14; 271-282; 337-346; 450-468 AND 656-664.
Tissue: Liver.
[2]"Genomic sequence of peroxisomal human branched chain acyl-CoA oxidase."
Fournier B., Baumgart E., Fahimi D., Mannaerts G.P., Van Veldhoven P.P.
Submitted (SEP-1997) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[3]"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S. expand/collapse author list , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
Tissue: Kidney.
[4]"The DNA sequence, annotation and analysis of human chromosome 3."
Muzny D.M., Scherer S.E., Kaul R., Wang J., Yu J., Sudbrak R., Buhay C.J., Chen R., Cree A., Ding Y., Dugan-Rocha S., Gill R., Gunaratne P., Harris R.A., Hawes A.C., Hernandez J., Hodgson A.V., Hume J. expand/collapse author list , Jackson A., Khan Z.M., Kovar-Smith C., Lewis L.R., Lozado R.J., Metzker M.L., Milosavljevic A., Miner G.R., Morgan M.B., Nazareth L.V., Scott G., Sodergren E., Song X.-Z., Steffen D., Wei S., Wheeler D.A., Wright M.W., Worley K.C., Yuan Y., Zhang Z., Adams C.Q., Ansari-Lari M.A., Ayele M., Brown M.J., Chen G., Chen Z., Clendenning J., Clerc-Blankenburg K.P., Chen R., Chen Z., Davis C., Delgado O., Dinh H.H., Dong W., Draper H., Ernst S., Fu G., Gonzalez-Garay M.L., Garcia D.K., Gillett W., Gu J., Hao B., Haugen E., Havlak P., He X., Hennig S., Hu S., Huang W., Jackson L.R., Jacob L.S., Kelly S.H., Kube M., Levy R., Li Z., Liu B., Liu J., Liu W., Lu J., Maheshwari M., Nguyen B.-V., Okwuonu G.O., Palmeiri A., Pasternak S., Perez L.M., Phelps K.A., Plopper F.J., Qiang B., Raymond C., Rodriguez R., Saenphimmachak C., Santibanez J., Shen H., Shen Y., Subramanian S., Tabor P.E., Verduzco D., Waldron L., Wang J., Wang J., Wang Q., Williams G.A., Wong G.K.-S., Yao Z., Zhang J., Zhang X., Zhao G., Zhou J., Zhou Y., Nelson D., Lehrach H., Reinhardt R., Naylor S.L., Yang H., Olson M., Weinstock G., Gibbs R.A.
Nature 440:1194-1198(2006) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[5]Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. expand/collapse author list , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., Venter J.C.
Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[6]"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
The MGC Project Team
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
Tissue: Colon.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
X95190 mRNA. Translation: CAA64489.1.
AJ001541 expand/collapse EMBL AC list , AJ001542, AJ001543, AJ001544, AJ001545, AJ001546, AJ001547, AJ001548 Genomic DNA. Translation: CAB65596.1.
AK313512 mRNA. Translation: BAG36292.1.
AC116036 Genomic DNA. No translation available.
CH471055 Genomic DNA. Translation: EAW65375.1.
BC047700 mRNA. Translation: AAH47700.1.
RefSeqNP_003491.1. NM_003500.3.
XP_005265562.1. XM_005265505.1.
UniGeneHs.444959.

3D structure databases

ProteinModelPortalQ99424.
SMRQ99424. Positions 18-671.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid113906. 1 interaction.
STRING9606.ENSP00000307697.

PTM databases

PhosphoSiteQ99424.

Polymorphism databases

DMDM17366636.

Proteomic databases

PaxDbQ99424.
PRIDEQ99424.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000302819; ENSP00000307697; ENSG00000168306.
GeneID8309.
KEGGhsa:8309.
UCSCuc003dkl.3. human.

Organism-specific databases

CTD8309.
GeneCardsGC03M058490.
HGNCHGNC:120. ACOX2.
HPAHPA038280.
MIM601641. gene.
neXtProtNX_Q99424.
PharmGKBPA24444.
GenAtlasSearch...

Phylogenomic databases

eggNOGCOG1960.
HOGENOMHOG000181256.
HOVERGENHBG050451.
InParanoidQ99424.
KOK10214.
OMAAFDFTDQ.
PhylomeDBQ99424.
TreeFamTF300672.

Enzyme and pathway databases

BioCycMetaCyc:HS09732-MONOMER.
ReactomeREACT_111217. Metabolism.

Gene expression databases

ArrayExpressQ99424.
BgeeQ99424.
CleanExHS_ACOX2.
GenevestigatorQ99424.

Family and domain databases

Gene3D1.10.540.10. 1 hit.
2.40.110.10. 1 hit.
InterProIPR006091. Acyl-CoA_Oxase/DH_cen-dom.
IPR012258. Acyl-CoA_oxidase.
IPR002655. Acyl-CoA_oxidase_C.
IPR009075. AcylCo_DH/oxidase_C.
IPR013786. AcylCoA_DH/ox_N.
IPR009100. AcylCoA_DH/oxidase_NM_dom.
[Graphical view]
PANTHERPTHR10909:SF11. PTHR10909:SF11. 1 hit.
PfamPF01756. ACOX. 1 hit.
PF02770. Acyl-CoA_dh_M. 1 hit.
[Graphical view]
PIRSFPIRSF000168. Acyl-CoA_oxidase. 1 hit.
SUPFAMSSF47203. SSF47203. 2 hits.
SSF56645. SSF56645. 1 hit.
ProtoNetSearch...

Other

GenomeRNAi8309.
NextBio31117.
PROQ99424.
SOURCESearch...

Entry information

Entry nameACOX2_HUMAN
AccessionPrimary (citable) accession number: Q99424
Secondary accession number(s): A6NF16, B2R8U5
Entry history
Integrated into UniProtKB/Swiss-Prot: November 2, 2001
Last sequence update: May 1, 1997
Last modified: April 16, 2014
This is version 129 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (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.

Relevant documents

SIMILARITY comments

Index of protein domains and families

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human chromosome 3

Human chromosome 3: entries, gene names and cross-references to MIM