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Protein

Prolyl 4-hydroxylase subunit alpha-3

Gene

P4HA3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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

Keywordsi

Molecular functionDioxygenase, Oxidoreductase
LigandIron, Metal-binding, Vitamin C

Enzyme and pathway databases

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

EuPathDBiHostDB:ENSG00000149380.11
HGNCiHGNC:30135 P4HA3
MIMi608987 gene
neXtProtiNX_Q7Z4N8

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

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...) asparagineSequence analysis1
Glycosylationi482N-linked (GlcNAc...) asparagineSequence analysis1

Post-translational modificationi

N-glycosylation plays no role in the catalytic activity.

Keywords - PTMi

Glycoprotein

Proteomic databases

MaxQBiQ7Z4N8
PaxDbiQ7Z4N8
PeptideAtlasiQ7Z4N8
PRIDEiQ7Z4N8
ProteomicsDBi69217
69218 [Q7Z4N8-2]

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

Show more details

Protein-protein interaction databases

BioGridi129495, 70 interactors
CORUMiQ7Z4N8
IntActiQ7Z4N8, 46 interactors
MINTiQ7Z4N8
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

Keywords - Domaini

Coiled coil, Signal, TPR repeat

Phylogenomic databases

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

Family and domain databases

Gene3Di1.25.40.10, 1 hit
InterProiView protein in InterPro
IPR005123 Oxoglu/Fe-dep_dioxygenase
IPR006620 Pro_4_hyd_alph
IPR013547 Pro_4_hyd_alph_N
IPR011990 TPR-like_helical_dom_sf
PfamiView protein in Pfam
PF13640 2OG-FeII_Oxy_3, 1 hit
PF08336 P4Ha_N, 1 hit
SMARTiView protein in SMART
SM00702 P4Hc, 1 hit
SUPFAMiSSF48452 SSF48452, 1 hit
PROSITEiView protein in PROSITE
PS51471 FE2OG_OXY, 1 hit

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 → N1 PublicationCorresponds to variant dbSNP:rs2282488Ensembl.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

Similar proteinsi

Entry informationi

Entry nameiP4HA3_HUMAN
AccessioniPrimary (citable) accession number: Q7Z4N8
Secondary accession number(s): A0AV13
, B4DUD3, Q5EBL3, Q5JPA9
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 5, 2008
Last sequence update: October 1, 2003
Last modified: July 18, 2018
This is version 136 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

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

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