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O15460 (P4HA2_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified April 16, 2014. Version 140. 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·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Prolyl 4-hydroxylase subunit alpha-2

Short name=4-PH alpha-2
EC=1.14.11.2
Alternative name(s):
Procollagen-proline,2-oxoglutarate-4-dioxygenase subunit alpha-2
Gene names
Name:P4HA2
ORF Names:UNQ290/PRO330
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length535 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.

Catalytic activity

L-proline-[procollagen] + 2-oxoglutarate + O2 = trans-4-hydroxy-L-proline-[procollagen] + succinate + CO2.

Cofactor

Binds 1 Fe2+ ion per subunit By similarity.

Ascorbate By similarity.

Subunit structure

Heterotetramer of two alpha-2 chains and two beta chains (the beta chain is the multi-functional PDI).

Subcellular location

Endoplasmic reticulum lumen.

Sequence similarities

Belongs to the P4HA family.

Contains 1 Fe2OG dioxygenase domain.

Contains 1 TPR repeat.

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]
Isoform IIb (identifier: O15460-1)

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.
Isoform IIa (identifier: O15460-2)

The sequence of this isoform differs from the canonical sequence as follows:
     436-451: NDERDTFKHLGTGNRV → RPFDSGLKTEGNRL

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Signal peptide1 – 2121 Potential
Chain22 – 535514Prolyl 4-hydroxylase subunit alpha-2
PRO_0000022726

Regions

Repeat207 – 24034TPR
Domain412 – 520109Fe2OG dioxygenase

Sites

Metal binding4301Iron By similarity
Metal binding4321Iron By similarity
Metal binding5011Iron By similarity
Binding site51112-oxoglutarate Potential

Amino acid modifications

Modified residue4801N6-succinyllysine By similarity
Glycosylation1151N-linked (GlcNAc...) Potential
Glycosylation2641N-linked (GlcNAc...) Potential

Natural variations

Alternative sequence436 – 45116NDERD…TGNRV → RPFDSGLKTEGNRL in isoform IIa.
VSP_004506

Sequences

Sequence LengthMass (Da)Tools
Isoform IIb [UniParc].

Last modified January 1, 1998. Version 1.
Checksum: FD04467B098F63CF

FASTA53560,902
        10         20         30         40         50         60 
MKLWVSALLM AWFGVLSCVQ AEFFTSIGHM TDLIYAEKEL VQSLKEYILV EEAKLSKIKS 

        70         80         90        100        110        120 
WANKMEALTS KSAADAEGYL AHPVNAYKLV KRLNTDWPAL EDLVLQDSAA GFIANLSVQR 

       130        140        150        160        170        180 
QFFPTDEDEI GAAKALMRLQ DTYRLDPGTI SRGELPGTKY QAMLSVDDCF GMGRSAYNEG 

       190        200        210        220        230        240 
DYYHTVLWME QVLKQLDAGE EATTTKSQVL DYLSYAVFQL GDLHRALELT RRLLSLDPSH 

       250        260        270        280        290        300 
ERAGGNLRYF EQLLEEEREK TLTNQTEAEL ATPEGIYERP VDYLPERDVY ESLCRGEGVK 

       310        320        330        340        350        360 
LTPRRQKRLF CRYHHGNRAP QLLIAPFKEE DEWDSPHIVR YYDVMSDEEI ERIKEIAKPK 

       370        380        390        400        410        420 
LARATVRDPK TGVLTVASYR VSKSSWLEED DDPVVARVNR RMQHITGLTV KTAELLQVAN 

       430        440        450        460        470        480 
YGVGGQYEPH FDFSRNDERD TFKHLGTGNR VATFLNYMSD VEAGGATVFP DLGAAIWPKK 

       490        500        510        520        530 
GTAVFWYNLL RSGEGDYRTR HAACPVLVGC KWVSNKWFHE RGQEFLRPCG STEVD 

« Hide

Isoform IIa [UniParc].

Checksum: 8C875AD482B0DBD2
Show »

FASTA53360,633

References

« Hide 'large scale' references
[1]"Cloning of the human prolyl 4-hydroxylase alpha subunit isoform alpha(II) and characterization of the type II enzyme tetramer. The alpha(I) and alpha(II) subunits do not form a mixed alpha(I)alpha(II)beta2 tetramer."
Annunen P., Helaakoski T., Myllyharju J., Veijola J., Pihlajaniemi T., Kivirikko K.I.
J. Biol. Chem. 272:17342-17348(1997) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM IIB).
Tissue: Lung.
[2]"Characterization of the human and mouse genes for the alpha subunit of type II prolyl 4-hydroxylase. Identification of a previously unknown alternatively spliced exon and its expression in various tissues."
Nokelainen M., Nissi R., Kukkola L., Helaakoski T., Myllyharju J.
Eur. J. Biochem. 268:5300-5309(2001) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE (ISOFORMS IIA AND IIB).
[3]"The secreted protein discovery initiative (SPDI), a large-scale effort to identify novel human secreted and transmembrane proteins: a bioinformatics assessment."
Clark H.F., Gurney A.L., Abaya E., Baker K., Baldwin D.T., Brush J., Chen J., Chow B., Chui C., Crowley C., Currell B., Deuel B., Dowd P., Eaton D., Foster J.S., Grimaldi C., Gu Q., Hass P.E. expand/collapse author list , Heldens S., Huang A., Kim H.S., Klimowski L., Jin Y., Johnson S., Lee J., Lewis L., Liao D., Mark M.R., Robbie E., Sanchez C., Schoenfeld J., Seshagiri S., Simmons L., Singh J., Smith V., Stinson J., Vagts A., Vandlen R.L., Watanabe C., Wieand D., Woods K., Xie M.-H., Yansura D.G., Yi S., Yu G., Yuan J., Zhang M., Zhang Z., Goddard A.D., Wood W.I., Godowski P.J., Gray A.M.
Genome Res. 13:2265-2270(2003) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM IIA).
[4]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 (SEP-2005) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[5]"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] (ISOFORM IIA).
Tissue: Brain.
[6]"Initial characterization of the human central proteome."
Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
U90441 mRNA. Translation: AAB71339.1.
AJ314859 Genomic DNA. Translation: CAC85688.1.
AJ314859 Genomic DNA. Translation: CAC85689.1.
AY358970 mRNA. Translation: AAQ89329.1.
CH471062 Genomic DNA. Translation: EAW62341.1.
CH471062 Genomic DNA. Translation: EAW62342.1.
CH471062 Genomic DNA. Translation: EAW62343.1.
CH471062 Genomic DNA. Translation: EAW62346.1.
BC035813 mRNA. Translation: AAH35813.1.
RefSeqNP_001017973.1. NM_001017973.1.
NP_001017974.1. NM_001017974.1.
NP_001136070.1. NM_001142598.1.
NP_001136071.1. NM_001142599.1.
NP_004190.1. NM_004199.2.
XP_005272173.1. XM_005272116.2.
XP_005272174.1. XM_005272117.2.
XP_005272175.1. XM_005272118.2.
XP_005272176.1. XM_005272119.2.
XP_005272177.1. XM_005272120.2.
UniGeneHs.519568.

3D structure databases

ProteinModelPortalO15460.
SMRO15460. Positions 23-257, 335-519.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid114464. 6 interactions.
IntActO15460. 7 interactions.
MINTMINT-1035479.
STRING9606.ENSP00000166534.

Chemistry

ChEMBLCHEMBL5640.
DrugBankDB00172. L-Proline.
DB00139. Succinic acid.

PTM databases

PhosphoSiteO15460.

Proteomic databases

PaxDbO15460.
PRIDEO15460.

Protocols and materials databases

DNASU8974.
StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000166534; ENSP00000166534; ENSG00000072682. [O15460-1]
ENST00000360568; ENSP00000353772; ENSG00000072682. [O15460-2]
ENST00000379086; ENSP00000368379; ENSG00000072682. [O15460-2]
ENST00000379100; ENSP00000368394; ENSG00000072682. [O15460-2]
ENST00000379104; ENSP00000368398; ENSG00000072682. [O15460-1]
ENST00000401867; ENSP00000384999; ENSG00000072682. [O15460-1]
GeneID8974.
KEGGhsa:8974.
UCSCuc003kwg.3. human. [O15460-2]
uc003kwh.3. human. [O15460-1]

Organism-specific databases

CTD8974.
GeneCardsGC05M131531.
HGNCHGNC:8547. P4HA2.
HPACAB062557.
HPA016997.
HPA027824.
MIM600608. gene.
neXtProtNX_O15460.
PharmGKBPA32875.
GenAtlasSearch...

Phylogenomic databases

eggNOGNOG78926.
HOGENOMHOG000230465.
HOVERGENHBG006834.
InParanoidO15460.
KOK00472.
OMACKWVSNK.
OrthoDBEOG7W6WKC.
PhylomeDBO15460.
TreeFamTF313393.

Enzyme and pathway databases

BRENDA1.14.11.2. 2681.

Gene expression databases

ArrayExpressO15460.
BgeeO15460.
CleanExHS_P4HA2.
GenevestigatorO15460.

Family and domain databases

Gene3D1.25.40.10. 2 hits.
InterProIPR005123. Oxoglu/Fe-dep_dioxygenase.
IPR006620. Pro_4_hyd_alph.
IPR013547. Pro_4_hyd_alph_N.
IPR013026. TPR-contain_dom.
IPR011990. TPR-like_helical.
IPR019734. TPR_repeat.
[Graphical view]
PfamPF03171. 2OG-FeII_Oxy. 1 hit.
PF08336. P4Ha_N. 1 hit.
[Graphical view]
SMARTSM00702. P4Hc. 1 hit.
[Graphical view]
PROSITEPS51471. FE2OG_OXY. 1 hit.
PS50005. TPR. 1 hit.
PS50293. TPR_REGION. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

GeneWikiP4HA2.
GenomeRNAi8974.
NextBio33677.
PROO15460.
SOURCESearch...

Entry information

Entry nameP4HA2_HUMAN
AccessionPrimary (citable) accession number: O15460
Secondary accession number(s): D3DQ85, D3DQ86, Q8WWN0
Entry history
Integrated into UniProtKB/Swiss-Prot: May 2, 2002
Last sequence update: January 1, 1998
Last modified: April 16, 2014
This is version 140 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 5

Human chromosome 5: entries, gene names and cross-references to MIM