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Q7Z4N8

- P4HA3_HUMAN

UniProt

Q7Z4N8 - P4HA3_HUMAN

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Protein
Prolyl 4-hydroxylase subunit alpha-3
Gene
P4HA3, UNQ711/PRO1374
Organism
Homo sapiens (Human)
Status
Reviewed - 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

Binds 1 Fe2+ ion per subunit.
Ascorbate.

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi440 – 4401Iron By similarity
Metal bindingi442 – 4421Iron By similarity
Metal bindingi510 – 5101Iron By similarity
Binding sitei520 – 52012-oxoglutarate Reviewed prediction

GO - Molecular functioni

  1. L-ascorbic acid binding Source: UniProtKB-KW
  2. iron ion binding Source: InterPro
  3. oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen Source: InterPro
  4. procollagen-proline 4-dioxygenase activity Source: UniProtKB-EC
Complete GO annotation...

Keywords - Molecular functioni

Dioxygenase, Oxidoreductase

Keywords - Ligandi

Iron, Metal-binding, Vitamin C

Enzyme and pathway databases

BRENDAi1.14.11.2. 2681.

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
ProteomesiUP000005640: Chromosome 11

Organism-specific databases

HGNCiHGNC:30135. P4HA3.

Subcellular locationi

GO - Cellular componenti

  1. endoplasmic reticulum lumen Source: UniProtKB-SubCell
Complete GO annotation...

Keywords - Cellular componenti

Endoplasmic reticulum

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA134870931.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Signal peptidei1 – 1919 Reviewed prediction
Add
BLAST
Chaini20 – 544525Prolyl 4-hydroxylase subunit alpha-3
PRO_0000317766Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Glycosylationi242 – 2421N-linked (GlcNAc...) Reviewed prediction
Glycosylationi482 – 4821N-linked (GlcNAc...) Reviewed prediction

Post-translational modificationi

N-glycosylation plays no role in the catalytic activity.

Keywords - PTMi

Glycoprotein

Proteomic databases

MaxQBiQ7Z4N8.
PaxDbiQ7Z4N8.
PRIDEiQ7Z4N8.

PTM databases

PhosphoSiteiQ7Z4N8.

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

ArrayExpressiQ7Z4N8.
BgeeiQ7Z4N8.
CleanExiHS_P4HA3.
GenevestigatoriQ7Z4N8.

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

Protein-protein interaction databases

BioGridi129495. 1 interaction.
STRINGi9606.ENSP00000332170.

Structurei

3D structure databases

ProteinModelPortaliQ7Z4N8.
SMRiQ7Z4N8. Positions 44-276, 338-528.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Repeati227 – 26034TPR
Add
BLAST
Domaini422 – 529108Fe2OG dioxygenase
Add
BLAST

Coiled coil

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Coiled coili107 – 13125 Reviewed prediction
Add
BLAST

Sequence similaritiesi

Belongs to the P4HA family.
Contains 1 TPR repeat.

Keywords - Domaini

Coiled coil, Signal, TPR repeat

Phylogenomic databases

eggNOGiNOG289769.
HOGENOMiHOG000230465.
HOVERGENiHBG006834.
InParanoidiQ7Z4N8.
KOiK00472.
OMAiHLEPYIA.
OrthoDBiEOG7W6WKC.
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.
[Graphical view]
PfamiPF03171. 2OG-FeII_Oxy. 1 hit.
PF08336. P4Ha_N. 1 hit.
[Graphical view]
SMARTiSM00702. P4Hc. 1 hit.
[Graphical view]
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. Align

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

MGPGARLAAL LAVLALGTGD PERAAARGDT FSALTSVARA LAPERRLLGL    50
LRRYLRGEEA RLRDLTRFYD KVLSLHEDST TPVANPLLAF TLIKRLQSDW 100
RNVVHSLEAS ENIRALKDGY EKVEQDLPAF EDLEGAARAL MRLQDVYMLN 150
VKGLARGVFQ RVTGSAITDL YSPKRLFSLT GDDCFQVGKV AYDMGDYYHA 200
IPWLEEAVSL FRGSYGEWKT EDEASLEDAL DHLAFAYFRA GNVSCALSLS 250
REFLLYSPDN KRMARNVLKY ERLLAESPNH VVAEAVIQRP NIPHLQTRDT 300
YEGLCQTLGS QPTLYQIPSL YCSYETNSNA YLLLQPIRKE VIHLEPYIAL 350
YHDFVSDSEA QKIRELAEPW LQRSVVASGE KQLQVEYRIS KSAWLKDTVD 400
PKLVTLNHRI AALTGLDVRP PYAEYLQVVN YGIGGHYEPH FDHATSPSSP 450
LYRMKSGNRV ATFMIYLSSV EAGGATAFIY ANLSVPVVRN AALFWWNLHR 500
SGEGDSDTLH AGCPVLVGDK WVANKWIHEY GQEFRRPCSS SPED 544
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

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti400 – 4001D → N.1 Publication
Corresponds to variant rs2282488 [ dbSNP | Ensembl ].
VAR_038675

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei257 – 2571S → R in isoform 2.
VSP_031146
Alternative sequencei258 – 544287Missing in isoform 2.
VSP_031147Add
BLAST
Alternative sequencei490 – 54455NAALF…SSPED → HCFGGTCTGVVKGTVTHFML AVLSWWEISGWPTSGYMSMD RNSADPAAPALKTELLAERS WWSPVAFQRSQEPKAGVGEE KAEQPPGRRPCQLCLCLANQ RQGRGCYQGTLRMYI in isoform 3.
VSP_054131Add
BLAST

Sequence conflict

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti222 – 2221D → Y in AAH89446. 1 Publication

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
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.
CCDSiCCDS8230.1. [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.
ENST00000524388; ENSP00000433860; ENSG00000149380. [Q7Z4N8-2]
GeneIDi283208.
KEGGihsa:283208.
UCSCiuc001ouz.3. human. [Q7Z4N8-1]

Polymorphism databases

DMDMi74738714.

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
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 CCDS8230.1. [Q7Z4N8-1 ]
RefSeqi NP_001275677.1. NM_001288748.1. [Q7Z4N8-3 ]
NP_878907.1. NM_182904.4. [Q7Z4N8-1 ]
UniGenei Hs.660541.

3D structure databases

ProteinModelPortali Q7Z4N8.
SMRi Q7Z4N8. Positions 44-276, 338-528.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

BioGridi 129495. 1 interaction.
STRINGi 9606.ENSP00000332170.

PTM databases

PhosphoSitei Q7Z4N8.

Polymorphism databases

DMDMi 74738714.

Proteomic databases

MaxQBi Q7Z4N8.
PaxDbi Q7Z4N8.
PRIDEi Q7Z4N8.

Protocols and materials databases

Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENST00000331597 ; ENSP00000332170 ; ENSG00000149380 . [Q7Z4N8-1 ]
ENST00000427714 ; ENSP00000401749 ; ENSG00000149380 .
ENST00000524388 ; ENSP00000433860 ; ENSG00000149380 . [Q7Z4N8-2 ]
GeneIDi 283208.
KEGGi hsa:283208.
UCSCi uc001ouz.3. human. [Q7Z4N8-1 ]

Organism-specific databases

CTDi 283208.
GeneCardsi GC11M073947.
HGNCi HGNC:30135. P4HA3.
HPAi HPA007897.
MIMi 608987. gene.
neXtProti NX_Q7Z4N8.
PharmGKBi PA134870931.
GenAtlasi Search...

Phylogenomic databases

eggNOGi NOG289769.
HOGENOMi HOG000230465.
HOVERGENi HBG006834.
InParanoidi Q7Z4N8.
KOi K00472.
OMAi HLEPYIA.
OrthoDBi EOG7W6WKC.
PhylomeDBi Q7Z4N8.
TreeFami TF313393.

Enzyme and pathway databases

BRENDAi 1.14.11.2. 2681.

Miscellaneous databases

ChiTaRSi P4HA3. human.
GenomeRNAii 283208.
NextBioi 35475155.
PROi Q7Z4N8.
SOURCEi Search...

Gene expression databases

ArrayExpressi Q7Z4N8.
Bgeei Q7Z4N8.
CleanExi HS_P4HA3.
Genevestigatori Q7Z4N8.

Family and domain databases

Gene3Di 1.25.40.10. 1 hit.
InterProi IPR005123. Oxoglu/Fe-dep_dioxygenase.
IPR006620. Pro_4_hyd_alph.
IPR013547. Pro_4_hyd_alph_N.
IPR011990. TPR-like_helical.
[Graphical view ]
Pfami PF03171. 2OG-FeII_Oxy. 1 hit.
PF08336. P4Ha_N. 1 hit.
[Graphical view ]
SMARTi SM00702. P4Hc. 1 hit.
[Graphical view ]
PROSITEi PS51471. FE2OG_OXY. 1 hit.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "Identification and characterization of a third human, rat, and mouse collagen prolyl 4-hydroxylase isoenzyme."
    Kukkola L., Hieta R., Kivirikko K.I., Myllyharju J.
    J. Biol. Chem. 278:47685-47693(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), FUNCTION, SUBUNIT, TISSUE SPECIFICITY.
    Tissue: Umbilical vein endothelial cell.
  2. "Cloning of a novel prolyl 4-hydroxylase subunit expressed in the fibrous cap of human atherosclerotic plaque."
    Van Den Diepstraten C., Papay K., Bolender Z., Brown A., Pickering J.G.
    Circulation 108:508-511(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), FUNCTION, SUBUNIT, TISSUE SPECIFICITY.
    Tissue: Vascular smooth muscle.
  3. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
  4. "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.
    , 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] (ISOFORM 3).
  5. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2).
    Tissue: Testis.
  6. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  7. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  8. "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 1), VARIANT ASN-400.
    Tissue: Colon.

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: July 9, 2014
This is version 104 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

External Data

Dasty 3

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