UniProtKB - Q7Z4N8 (P4HA3_HUMAN)
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Protein
Prolyl 4-hydroxylase subunit alpha-3
Gene
P4HA3
Organism
Homo sapiens (Human)
Status
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:- Fe2+Note: Binds 1 Fe2+ ion per subunit.
- L-ascorbate
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Metal bindingi | 440 | IronPROSITE-ProRule annotation | 1 | |
Metal bindingi | 442 | IronPROSITE-ProRule annotation | 1 | |
Metal bindingi | 510 | IronPROSITE-ProRule annotation | 1 | |
Binding sitei | 520 | 2-oxoglutaratePROSITE-ProRule annotation | 1 |
GO - Molecular functioni
- iron ion binding Source: InterPro
- L-ascorbic acid binding Source: UniProtKB-KW
- oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen Source: InterPro
- procollagen-proline 4-dioxygenase activity Source: UniProtKB-EC
Keywordsi
Molecular function | Dioxygenase, Oxidoreductase |
Ligand | Iron, Metal-binding, Vitamin C |
Enzyme and pathway databases
BRENDAi | 1.14.11.2. 2681. |
Reactomei | R-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 |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
EuPathDBi | HostDB:ENSG00000149380.11. |
HGNCi | HGNC:30135. P4HA3. |
MIMi | 608987. gene. |
neXtProti | NX_Q7Z4N8. |
Pathology & Biotechi
Organism-specific databases
DisGeNETi | 283208. |
OpenTargetsi | ENSG00000149380. |
PharmGKBi | PA134870931. |
Polymorphism and mutation databases
BioMutai | P4HA3. |
DMDMi | 74738714. |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Signal peptidei | 1 – 19 | Sequence analysisAdd BLAST | 19 | |
ChainiPRO_0000317766 | 20 – 544 | Prolyl 4-hydroxylase subunit alpha-3Add BLAST | 525 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Glycosylationi | 242 | N-linked (GlcNAc...) asparagineSequence analysis | 1 | |
Glycosylationi | 482 | N-linked (GlcNAc...) asparagineSequence analysis | 1 |
Post-translational modificationi
N-glycosylation plays no role in the catalytic activity.
Keywords - PTMi
GlycoproteinProteomic databases
MaxQBi | Q7Z4N8. |
PaxDbi | Q7Z4N8. |
PeptideAtlasi | Q7Z4N8. |
PRIDEi | Q7Z4N8. |
PTM databases
iPTMneti | Q7Z4N8. |
PhosphoSitePlusi | Q7Z4N8. |
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
Bgeei | ENSG00000149380. |
CleanExi | HS_P4HA3. |
ExpressionAtlasi | Q7Z4N8. baseline and differential. |
Genevisiblei | Q7Z4N8. HS. |
Organism-specific databases
HPAi | HPA007897. |
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
Protein-protein interaction databases
BioGridi | 129495. 70 interactors. |
CORUMi | Q7Z4N8. |
IntActi | Q7Z4N8. 28 interactors. |
MINTi | Q7Z4N8. |
STRINGi | 9606.ENSP00000332170. |
Structurei
3D structure databases
ProteinModelPortali | Q7Z4N8. |
SMRi | Q7Z4N8. |
ModBasei | Search... |
MobiDBi | Search... |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Repeati | 227 – 260 | TPRAdd BLAST | 34 | |
Domaini | 422 – 529 | Fe2OG dioxygenasePROSITE-ProRule annotationAdd BLAST | 108 |
Coiled coil
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Coiled coili | 107 – 131 | Sequence analysisAdd BLAST | 25 |
Sequence similaritiesi
Belongs to the P4HA family.Curated
Keywords - Domaini
Coiled coil, Signal, TPR repeatPhylogenomic databases
eggNOGi | KOG1591. Eukaryota. ENOG410XS5J. LUCA. |
GeneTreei | ENSGT00390000018885. |
HOGENOMi | HOG000230465. |
HOVERGENi | HBG006834. |
InParanoidi | Q7Z4N8. |
KOi | K00472. |
OMAi | LQSDWRN. |
OrthoDBi | EOG091G0749. |
PhylomeDBi | Q7Z4N8. |
TreeFami | TF313393. |
Family and domain databases
Gene3Di | 1.25.40.10. 1 hit. |
InterProi | View 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. |
Pfami | View protein in Pfam PF13640. 2OG-FeII_Oxy_3. 1 hit. PF08336. P4Ha_N. 1 hit. |
SMARTi | View protein in SMART SM00702. P4Hc. 1 hit. |
SUPFAMi | SSF48452. SSF48452. 1 hit. |
PROSITEi | View protein in PROSITE PS51471. FE2OG_OXY. 1 hit. |
s (3)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 3 produced by isoformsialternative 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.
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
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 222 | D → Y in AAH89446 (PubMed:15489334).Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_038675 | 400 | D → N1 PublicationCorresponds to variant dbSNP:rs2282488Ensembl. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_031146 | 257 | S → R in isoform 2. 1 Publication | 1 | |
Alternative sequenceiVSP_031147 | 258 – 544 | Missing in isoform 2. 1 PublicationAdd BLAST | 287 | |
Alternative sequenceiVSP_054131 | 490 – 544 | NAALF…SSPED → HCFGGTCTGVVKGTVTHFML AVLSWWEISGWPTSGYMSMD RNSADPAAPALKTELLAERS WWSPVAFQRSQEPKAGVGEE KAEQPPGRRPCQLCLCLANQ RQGRGCYQGTLRMYI in isoform 3. 1 PublicationAdd BLAST | 55 |
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. |
CCDSi | CCDS73347.1. [Q7Z4N8-3] CCDS8230.1. [Q7Z4N8-1] |
RefSeqi | NP_001275677.1. NM_001288748.1. [Q7Z4N8-3] NP_878907.1. NM_182904.4. [Q7Z4N8-1] |
UniGenei | Hs.660541. |
Genome annotation databases
Ensembli | ENST00000331597; ENSP00000332170; ENSG00000149380. [Q7Z4N8-1] ENST00000427714; ENSP00000401749; ENSG00000149380. [Q7Z4N8-3] ENST00000524388; ENSP00000433860; ENSG00000149380. [Q7Z4N8-2] |
GeneIDi | 283208. |
KEGGi | hsa:283208. |
UCSCi | uc001ouz.5. human. [Q7Z4N8-1] |
Keywords - Coding sequence diversityi
Alternative splicing, PolymorphismSimilar proteinsi
Entry informationi
Entry namei | P4HA3_HUMAN | |
Accessioni | Q7Z4N8Primary (citable) accession number: Q7Z4N8 Secondary accession number(s): A0AV13 Q5JPA9 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | February 5, 2008 |
Last sequence update: | October 1, 2003 | |
Last modified: | March 28, 2018 | |
This is version 134 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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. |