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Protein

Prolyl 4-hydroxylase subunit alpha-3

Gene

P4HA3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.2 Publications

Catalytic activityi

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

Cofactori

Protein has several cofactor binding sites:

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi440IronPROSITE-ProRule annotation1
Metal bindingi442IronPROSITE-ProRule annotation1
Metal bindingi510IronPROSITE-ProRule annotation1
Binding sitei5202-oxoglutaratePROSITE-ProRule annotation1

GO - Molecular functioni

Complete GO annotation...

Keywords - Molecular functioni

Dioxygenase, Oxidoreductase

Keywords - Ligandi

Iron, Metal-binding, Vitamin C

Enzyme and pathway databases

BioCyciZFISH:HS14281-MONOMER.
BRENDAi1.14.11.2. 2681.
ReactomeiR-HSA-1650814. Collagen biosynthesis and modifying enzymes.

Names & Taxonomyi

Protein namesi
Recommended name:
Prolyl 4-hydroxylase subunit alpha-3 (EC:1.14.11.2)
Short name:
4-PH alpha-3
Alternative name(s):
Procollagen-proline,2-oxoglutarate-4-dioxygenase subunit alpha-3
Gene namesi
Name:P4HA3
ORF Names:UNQ711/PRO1374
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 11

Organism-specific databases

HGNCiHGNC:30135. P4HA3.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Endoplasmic reticulum

Pathology & Biotechi

Organism-specific databases

DisGeNETi283208.
OpenTargetsiENSG00000149380.
PharmGKBiPA134870931.

Polymorphism and mutation databases

BioMutaiP4HA3.
DMDMi74738714.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 19Sequence analysisAdd BLAST19
ChainiPRO_000031776620 – 544Prolyl 4-hydroxylase subunit alpha-3Add BLAST525

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi242N-linked (GlcNAc...)Sequence analysis1
Glycosylationi482N-linked (GlcNAc...)Sequence analysis1

Post-translational modificationi

N-glycosylation plays no role in the catalytic activity.

Keywords - PTMi

Glycoprotein

Proteomic databases

EPDiQ7Z4N8.
MaxQBiQ7Z4N8.
PaxDbiQ7Z4N8.
PeptideAtlasiQ7Z4N8.
PRIDEiQ7Z4N8.

PTM databases

iPTMnetiQ7Z4N8.
PhosphoSitePlusiQ7Z4N8.

Expressioni

Tissue specificityi

Highly expressed in placenta, liver and fetal skin. Weakly expressed in fetal epiphyseal cartilage, fetal liver, fibroblast, lung and skeletal muscle. Expressed also in fibrous cap of carotid atherosclerotic lesions.2 Publications

Gene expression databases

BgeeiENSG00000149380.
CleanExiHS_P4HA3.
ExpressionAtlasiQ7Z4N8. baseline and differential.
GenevisibleiQ7Z4N8. HS.

Organism-specific databases

HPAiHPA007897.

Interactioni

Subunit structurei

Heterotetramer of two alpha-3 chains and two beta chains (the beta chain is the multi-functional PDI).2 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
DTNBP1Q96EV83EBI-10181968,EBI-465804
HGSO149645EBI-10181968,EBI-740220
MAGEA2BP433564EBI-10181968,EBI-5650739
MAGEA2BQ96E033EBI-10181968,EBI-10239285
MAGEC2Q9UBF13EBI-10181968,EBI-5651487
MEOX2A4D1273EBI-10181968,EBI-10172134
NUTM1Q86Y263EBI-10181968,EBI-10178410
SERTAD1Q53GC03EBI-10181968,EBI-2826300
WDR62O433793EBI-10181968,EBI-714790
ZC3H12AQ5D1E85EBI-10181968,EBI-747793

Protein-protein interaction databases

BioGridi129495. 65 interactors.
IntActiQ7Z4N8. 15 interactors.
STRINGi9606.ENSP00000332170.

Structurei

3D structure databases

ProteinModelPortaliQ7Z4N8.
SMRiQ7Z4N8.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Repeati227 – 260TPRAdd BLAST34
Domaini422 – 529Fe2OG dioxygenasePROSITE-ProRule annotationAdd BLAST108

Coiled coil

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Coiled coili107 – 131Sequence analysisAdd BLAST25

Sequence similaritiesi

Belongs to the P4HA family.Curated
Contains 1 Fe2OG dioxygenase domain.PROSITE-ProRule annotation
Contains 1 TPR repeat.Curated

Keywords - Domaini

Coiled coil, Signal, TPR repeat

Phylogenomic databases

eggNOGiKOG1591. Eukaryota.
ENOG410XS5J. LUCA.
GeneTreeiENSGT00390000018885.
HOGENOMiHOG000230465.
HOVERGENiHBG006834.
InParanoidiQ7Z4N8.
KOiK00472.
OMAiHLEPYIA.
OrthoDBiEOG091G0749.
PhylomeDBiQ7Z4N8.
TreeFamiTF313393.

Family and domain databases

Gene3Di1.25.40.10. 1 hit.
InterProiIPR005123. Oxoglu/Fe-dep_dioxygenase.
IPR006620. Pro_4_hyd_alph.
IPR013547. Pro_4_hyd_alph_N.
IPR011990. TPR-like_helical_dom.
[Graphical view]
PfamiPF13640. 2OG-FeII_Oxy_3. 1 hit.
PF08336. P4Ha_N. 1 hit.
[Graphical view]
SMARTiSM00702. P4Hc. 1 hit.
[Graphical view]
SUPFAMiSSF48452. SSF48452. 1 hit.
PROSITEiPS51471. FE2OG_OXY. 1 hit.
[Graphical view]

Sequences (3)i

Sequence statusi: Complete.

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

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q7Z4N8-1) [UniParc]FASTAAdd to basket

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.

« Hide

        10         20         30         40         50
MGPGARLAAL LAVLALGTGD PERAAARGDT FSALTSVARA LAPERRLLGL
60 70 80 90 100
LRRYLRGEEA RLRDLTRFYD KVLSLHEDST TPVANPLLAF TLIKRLQSDW
110 120 130 140 150
RNVVHSLEAS ENIRALKDGY EKVEQDLPAF EDLEGAARAL MRLQDVYMLN
160 170 180 190 200
VKGLARGVFQ RVTGSAITDL YSPKRLFSLT GDDCFQVGKV AYDMGDYYHA
210 220 230 240 250
IPWLEEAVSL FRGSYGEWKT EDEASLEDAL DHLAFAYFRA GNVSCALSLS
260 270 280 290 300
REFLLYSPDN KRMARNVLKY ERLLAESPNH VVAEAVIQRP NIPHLQTRDT
310 320 330 340 350
YEGLCQTLGS QPTLYQIPSL YCSYETNSNA YLLLQPIRKE VIHLEPYIAL
360 370 380 390 400
YHDFVSDSEA QKIRELAEPW LQRSVVASGE KQLQVEYRIS KSAWLKDTVD
410 420 430 440 450
PKLVTLNHRI AALTGLDVRP PYAEYLQVVN YGIGGHYEPH FDHATSPSSP
460 470 480 490 500
LYRMKSGNRV ATFMIYLSSV EAGGATAFIY ANLSVPVVRN AALFWWNLHR
510 520 530 540
SGEGDSDTLH AGCPVLVGDK WVANKWIHEY GQEFRRPCSS SPED
Length:544
Mass (Da):61,126
Last modified:October 1, 2003 - v1
Checksum:i1C88C168A268572F
GO
Isoform 2 (identifier: Q7Z4N8-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     257-257: S → R
     258-544: Missing.

Note: No experimental confirmation available.
Show »
Length:257
Mass (Da):28,701
Checksum:iA610B9B8CF4E4238
GO
Isoform 3 (identifier: Q7Z4N8-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     490-544: NAALFWWNLH...RRPCSSSPED → HCFGGTCTGV...CYQGTLRMYI

Note: No experimental confirmation available.
Show »
Length:604
Mass (Da):67,591
Checksum:iE2A37A126C60A2A0
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti222D → Y in AAH89446 (PubMed:15489334).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_038675400D → N.1 PublicationCorresponds to variant rs2282488dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_031146257S → R in isoform 2. 1 Publication1
Alternative sequenceiVSP_031147258 – 544Missing in isoform 2. 1 PublicationAdd BLAST287
Alternative sequenceiVSP_054131490 – 544NAALF…SSPED → HCFGGTCTGVVKGTVTHFML AVLSWWEISGWPTSGYMSMD RNSADPAAPALKTELLAERS WWSPVAFQRSQEPKAGVGEE KAEQPPGRRPCQLCLCLANQ RQGRGCYQGTLRMYI in isoform 3. 1 PublicationAdd BLAST55

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY313448 mRNA. Translation: AAQ87603.1.
AY327887 mRNA. Translation: AAP97874.1.
AY358521 mRNA. Translation: AAQ88885.1.
AK300598 mRNA. Translation: BAG62295.1.
AP000577 Genomic DNA. No translation available.
AP001085 Genomic DNA. No translation available.
AL833965 mRNA. Translation: CAI46215.1.
CH471076 Genomic DNA. Translation: EAW74935.1.
BC089446 mRNA. Translation: AAH89446.1.
BC117333 mRNA. Translation: AAI17334.1.
BC126170 mRNA. Translation: AAI26171.1.
CCDSiCCDS73347.1. [Q7Z4N8-3]
CCDS8230.1. [Q7Z4N8-1]
RefSeqiNP_001275677.1. NM_001288748.1. [Q7Z4N8-3]
NP_878907.1. NM_182904.4. [Q7Z4N8-1]
UniGeneiHs.660541.

Genome annotation databases

EnsembliENST00000331597; ENSP00000332170; ENSG00000149380. [Q7Z4N8-1]
ENST00000427714; ENSP00000401749; ENSG00000149380. [Q7Z4N8-3]
ENST00000524388; ENSP00000433860; ENSG00000149380. [Q7Z4N8-2]
GeneIDi283208.
KEGGihsa:283208.
UCSCiuc001ouz.5. human. [Q7Z4N8-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY313448 mRNA. Translation: AAQ87603.1.
AY327887 mRNA. Translation: AAP97874.1.
AY358521 mRNA. Translation: AAQ88885.1.
AK300598 mRNA. Translation: BAG62295.1.
AP000577 Genomic DNA. No translation available.
AP001085 Genomic DNA. No translation available.
AL833965 mRNA. Translation: CAI46215.1.
CH471076 Genomic DNA. Translation: EAW74935.1.
BC089446 mRNA. Translation: AAH89446.1.
BC117333 mRNA. Translation: AAI17334.1.
BC126170 mRNA. Translation: AAI26171.1.
CCDSiCCDS73347.1. [Q7Z4N8-3]
CCDS8230.1. [Q7Z4N8-1]
RefSeqiNP_001275677.1. NM_001288748.1. [Q7Z4N8-3]
NP_878907.1. NM_182904.4. [Q7Z4N8-1]
UniGeneiHs.660541.

3D structure databases

ProteinModelPortaliQ7Z4N8.
SMRiQ7Z4N8.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi129495. 65 interactors.
IntActiQ7Z4N8. 15 interactors.
STRINGi9606.ENSP00000332170.

PTM databases

iPTMnetiQ7Z4N8.
PhosphoSitePlusiQ7Z4N8.

Polymorphism and mutation databases

BioMutaiP4HA3.
DMDMi74738714.

Proteomic databases

EPDiQ7Z4N8.
MaxQBiQ7Z4N8.
PaxDbiQ7Z4N8.
PeptideAtlasiQ7Z4N8.
PRIDEiQ7Z4N8.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000331597; ENSP00000332170; ENSG00000149380. [Q7Z4N8-1]
ENST00000427714; ENSP00000401749; ENSG00000149380. [Q7Z4N8-3]
ENST00000524388; ENSP00000433860; ENSG00000149380. [Q7Z4N8-2]
GeneIDi283208.
KEGGihsa:283208.
UCSCiuc001ouz.5. human. [Q7Z4N8-1]

Organism-specific databases

CTDi283208.
DisGeNETi283208.
GeneCardsiP4HA3.
HGNCiHGNC:30135. P4HA3.
HPAiHPA007897.
MIMi608987. gene.
neXtProtiNX_Q7Z4N8.
OpenTargetsiENSG00000149380.
PharmGKBiPA134870931.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1591. Eukaryota.
ENOG410XS5J. LUCA.
GeneTreeiENSGT00390000018885.
HOGENOMiHOG000230465.
HOVERGENiHBG006834.
InParanoidiQ7Z4N8.
KOiK00472.
OMAiHLEPYIA.
OrthoDBiEOG091G0749.
PhylomeDBiQ7Z4N8.
TreeFamiTF313393.

Enzyme and pathway databases

BioCyciZFISH:HS14281-MONOMER.
BRENDAi1.14.11.2. 2681.
ReactomeiR-HSA-1650814. Collagen biosynthesis and modifying enzymes.

Miscellaneous databases

ChiTaRSiP4HA3. human.
GenomeRNAii283208.
PROiQ7Z4N8.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000149380.
CleanExiHS_P4HA3.
ExpressionAtlasiQ7Z4N8. baseline and differential.
GenevisibleiQ7Z4N8. HS.

Family and domain databases

Gene3Di1.25.40.10. 1 hit.
InterProiIPR005123. Oxoglu/Fe-dep_dioxygenase.
IPR006620. Pro_4_hyd_alph.
IPR013547. Pro_4_hyd_alph_N.
IPR011990. TPR-like_helical_dom.
[Graphical view]
PfamiPF13640. 2OG-FeII_Oxy_3. 1 hit.
PF08336. P4Ha_N. 1 hit.
[Graphical view]
SMARTiSM00702. P4Hc. 1 hit.
[Graphical view]
SUPFAMiSSF48452. SSF48452. 1 hit.
PROSITEiPS51471. FE2OG_OXY. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiP4HA3_HUMAN
AccessioniPrimary (citable) accession number: Q7Z4N8
Secondary accession number(s): A0AV13
, B4DUD3, Q5EBL3, Q5JPA9
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 5, 2008
Last sequence update: October 1, 2003
Last modified: November 30, 2016
This is version 124 of the entry and version 1 of the sequence. [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

Complete proteome, Reference proteome

Documents

  1. Human chromosome 11
    Human chromosome 11: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.