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Protein

Prolyl 4-hydroxylase subunit alpha-2

Gene

P4ha2

Organism
Mus musculus (Mouse)
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.

Catalytic activityi

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

Cofactori

Protein has several cofactor binding sites:

Enzyme regulationi

In contrast to the subunit alpha-1, alpha-2 is inhibited by poly(L-proline) only at very high concentrations.

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi432 – 4321IronPROSITE-ProRule annotation
Metal bindingi434 – 4341IronPROSITE-ProRule annotation
Metal bindingi503 – 5031IronPROSITE-ProRule annotation
Binding sitei513 – 51312-oxoglutaratePROSITE-ProRule annotation

GO - Molecular functioni

GO - Biological processi

  • peptidyl-proline hydroxylation to 4-hydroxy-L-proline Source: MGI
Complete GO annotation...

Keywords - Molecular functioni

Dioxygenase, Oxidoreductase

Keywords - Ligandi

Iron, Metal-binding, Vitamin C

Names & Taxonomyi

Protein namesi
Recommended name:
Prolyl 4-hydroxylase subunit alpha-2 (EC:1.14.11.2)
Short name:
4-PH alpha-2
Alternative name(s):
Procollagen-proline,2-oxoglutarate-4-dioxygenase subunit alpha-2
Gene namesi
Name:P4ha2
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 11

Organism-specific databases

MGIiMGI:894286. P4ha2.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Endoplasmic reticulum

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Signal peptidei1 – 2323Sequence analysisAdd
BLAST
Chaini24 – 537514Prolyl 4-hydroxylase subunit alpha-2PRO_0000022727Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Glycosylationi117 – 1171N-linked (GlcNAc...)Sequence analysis
Glycosylationi266 – 2661N-linked (GlcNAc...)Sequence analysis
Modified residuei482 – 4821N6-succinyllysineCombined sources

Keywords - PTMi

Glycoprotein

Proteomic databases

EPDiQ60716.
MaxQBiQ60716.
PaxDbiQ60716.
PRIDEiQ60716.

PTM databases

PhosphoSiteiQ60716.

Expressioni

Tissue specificityi

Expressed in a variety of tissues.

Gene expression databases

BgeeiQ60716.
ExpressionAtlasiQ60716. baseline and differential.
GenevisibleiQ60716. MM.

Interactioni

Subunit structurei

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

Protein-protein interaction databases

BioGridi202007. 2 interactions.
IntActiQ60716. 2 interactions.
MINTiMINT-4106103.
STRINGi10090.ENSMUSP00000019050.

Structurei

3D structure databases

ProteinModelPortaliQ60716.
SMRiQ60716. Positions 25-259, 337-521.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Repeati209 – 24234TPRAdd
BLAST
Domaini414 – 522109Fe2OG dioxygenasePROSITE-ProRule annotationAdd
BLAST

Sequence similaritiesi

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

Keywords - Domaini

Signal, TPR repeat

Phylogenomic databases

eggNOGiKOG1591. Eukaryota.
ENOG410XS5J. LUCA.
GeneTreeiENSGT00390000018885.
HOGENOMiHOG000230465.
HOVERGENiHBG006834.
InParanoidiQ60716.
KOiK00472.
PhylomeDBiQ60716.

Family and domain databases

Gene3Di1.25.40.10. 2 hits.
InterProiIPR005123. Oxoglu/Fe-dep_dioxygenase.
IPR006620. Pro_4_hyd_alph.
IPR013547. Pro_4_hyd_alph_N.
IPR013026. TPR-contain_dom.
IPR011990. TPR-like_helical_dom.
IPR019734. TPR_repeat.
[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.
PS50005. TPR. 1 hit.
PS50293. TPR_REGION. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform IIb (identifier: Q60716-1) [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
MKLQVLVLVL LMSWFGVLSW VQAEFFTSIG HMTDLIYAEK DLVQSLKEYI
60 70 80 90 100
LVEEAKLAKI KSWASKMEAL TSRSAADPEG YLAHPVNAYK LVKRLNTDWP
110 120 130 140 150
ALGDLVLQDA SAGFVANLSV QRQFFPTDED ESGAARALMR LQDTYKLDPD
160 170 180 190 200
TISRGELPGT KYQAMLSVDD CFGLGRSAYN EGDYYHTVLW MEQVLKQLDA
210 220 230 240 250
GEEATVTKSL VLDYLSYAVF QLGDLHRAVE LTRRLLSLDP SHERAGGNLR
260 270 280 290 300
YFERLLEEER GKSLSNQTDA GLATQENLYE RPTDYLPERD VYESLCRGEG
310 320 330 340 350
VKLTPRRQKK LFCRYHHGNR VPQLLIAPFK EEDEWDSPHI VRYYDVMSDE
360 370 380 390 400
EIERIKEIAK PKLARATVRD PKTGVLTVAS YRVSKSSWLE EDDDPVVARV
410 420 430 440 450
NRRMQHITGL TVKTAELLQV ANYGMGGQYE PHFDFSRSDD EDAFKRLGTG
460 470 480 490 500
NRVATFLNYM SDVEAGGATV FPDLGAAIWP KKGTAVFWYN LLRSGEGDYR
510 520 530
TRHAACPVLV GCKWVSNKWF HERGQEFLRP CGTTEVD
Length:537
Mass (Da):61,002
Last modified:November 1, 1996 - v1
Checksum:iC47D976C75E82518
GO
Isoform IIa (identifier: Q60716-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     438-453: SDDEDAFKRLGTGNRV → RPFDSGLKTEGNRL

Show »
Length:535
Mass (Da):60,812
Checksum:iBBA7883CD43CB996
GO

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei438 – 45316SDDED…TGNRV → RPFDSGLKTEGNRL in isoform IIa. 1 PublicationVSP_004507Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U16163 mRNA. Translation: AAC52198.1.
AJ314858 Genomic DNA. Translation: CAC85690.1.
AJ314858 Genomic DNA. Translation: CAC85691.1.
BC018411 mRNA. Translation: AAH18411.1.
CCDSiCCDS24691.1. [Q60716-1]
CCDS48797.1. [Q60716-2]
PIRiI49135.
RefSeqiNP_001129548.1. NM_001136076.2. [Q60716-2]
NP_035161.2. NM_011031.2.
UniGeneiMm.3705.

Genome annotation databases

EnsembliENSMUST00000093107; ENSMUSP00000091749; ENSMUSG00000018906. [Q60716-2]
ENSMUST00000174616; ENSMUSP00000133275; ENSMUSG00000018906. [Q60716-2]
GeneIDi18452.
KEGGimmu:18452.

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U16163 mRNA. Translation: AAC52198.1.
AJ314858 Genomic DNA. Translation: CAC85690.1.
AJ314858 Genomic DNA. Translation: CAC85691.1.
BC018411 mRNA. Translation: AAH18411.1.
CCDSiCCDS24691.1. [Q60716-1]
CCDS48797.1. [Q60716-2]
PIRiI49135.
RefSeqiNP_001129548.1. NM_001136076.2. [Q60716-2]
NP_035161.2. NM_011031.2.
UniGeneiMm.3705.

3D structure databases

ProteinModelPortaliQ60716.
SMRiQ60716. Positions 25-259, 337-521.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi202007. 2 interactions.
IntActiQ60716. 2 interactions.
MINTiMINT-4106103.
STRINGi10090.ENSMUSP00000019050.

PTM databases

PhosphoSiteiQ60716.

Proteomic databases

EPDiQ60716.
MaxQBiQ60716.
PaxDbiQ60716.
PRIDEiQ60716.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000093107; ENSMUSP00000091749; ENSMUSG00000018906. [Q60716-2]
ENSMUST00000174616; ENSMUSP00000133275; ENSMUSG00000018906. [Q60716-2]
GeneIDi18452.
KEGGimmu:18452.

Organism-specific databases

CTDi8974.
MGIiMGI:894286. P4ha2.

Phylogenomic databases

eggNOGiKOG1591. Eukaryota.
ENOG410XS5J. LUCA.
GeneTreeiENSGT00390000018885.
HOGENOMiHOG000230465.
HOVERGENiHBG006834.
InParanoidiQ60716.
KOiK00472.
PhylomeDBiQ60716.

Miscellaneous databases

NextBioi294140.
PROiQ60716.
SOURCEiSearch...

Gene expression databases

BgeeiQ60716.
ExpressionAtlasiQ60716. baseline and differential.
GenevisibleiQ60716. MM.

Family and domain databases

Gene3Di1.25.40.10. 2 hits.
InterProiIPR005123. Oxoglu/Fe-dep_dioxygenase.
IPR006620. Pro_4_hyd_alph.
IPR013547. Pro_4_hyd_alph_N.
IPR013026. TPR-contain_dom.
IPR011990. TPR-like_helical_dom.
IPR019734. TPR_repeat.
[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.
PS50005. TPR. 1 hit.
PS50293. TPR_REGION. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "Cloning, baculovirus expression, and characterization of a second mouse prolyl 4-hydroxylase alpha-subunit isoform: formation of an alpha 2 beta 2 tetramer with the protein disulfide-isomerase/beta subunit."
    Helaakoski T., Annunen P., Vuori K., Macneil I.A., Pihlajaniemi T., Kivirikko K.I.
    Proc. Natl. Acad. Sci. U.S.A. 92:4427-4431(1995) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM IIB).
  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 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).
  4. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Heart and Lung.
  5. "SIRT5-mediated lysine desuccinylation impacts diverse metabolic pathways."
    Park J., Chen Y., Tishkoff D.X., Peng C., Tan M., Dai L., Xie Z., Zhang Y., Zwaans B.M., Skinner M.E., Lombard D.B., Zhao Y.
    Mol. Cell 50:919-930(2013) [PubMed] [Europe PMC] [Abstract]
    Cited for: SUCCINYLATION [LARGE SCALE ANALYSIS] AT LYS-482, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Embryonic fibroblast.

Entry informationi

Entry nameiP4HA2_MOUSE
AccessioniPrimary (citable) accession number: Q60716
Secondary accession number(s): Q8VBU4
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: November 1, 1996
Last modified: March 16, 2016
This is version 139 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. 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.