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Protein

Prolyl 3-hydroxylase 2

Gene

P3h2

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Prolyl 3-hydroxylase that catalyzes the post-translational formation of 3-hydroxyproline on collagens (PubMed:24368846, PubMed:25645914). Contributes to proline 3-hydroxylation of collagen COL4A1 and COL1A1 in tendons, the eye sclera and in the eye lens capsule (PubMed:25645914). Has high activity with the type IV collagen COL4A1, and lower activity with COL1A1. Catalyzes hydroxylation of the first Pro in Gly-Pro-Hyp sequences where Hyp is 4-hydroxyproline. Has no activity on substrates that have proline instead of 4-hydroxyproline in the third position (By similarity).By similarity2 Publications

Catalytic activityi

L-proline-[procollagen] + 2-oxoglutarate + O2 = trans-3-hydroxy-L-proline-[procollagen] + succinate + CO2.1 Publication

Cofactori

Protein has several cofactor binding sites:
  • Fe cationBy similarity
  • L-ascorbateBy similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi575Iron1
Metal bindingi577Iron1
Metal bindingi647Iron1
Active sitei657By similarity1

GO - Molecular functioni

  • iron ion binding Source: InterPro
  • L-ascorbic acid binding Source: UniProtKB-KW
  • procollagen-proline 3-dioxygenase activity Source: UniProtKB

GO - Biological processi

  • collagen metabolic process Source: UniProtKB
  • negative regulation of cell proliferation Source: UniProtKB
  • peptidyl-proline hydroxylation Source: UniProtKB

Keywordsi

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

Enzyme and pathway databases

BRENDAi1.14.11.7 3474
ReactomeiR-MMU-1650814 Collagen biosynthesis and modifying enzymes

Names & Taxonomyi

Protein namesi
Recommended name:
Prolyl 3-hydroxylase 2Imported (EC:1.14.11.71 Publication)
Alternative name(s):
Leprecan-like protein 11 Publication
Gene namesi
Name:P3h2Imported
Synonyms:Leprel11 Publication
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 16

Organism-specific databases

MGIiMGI:2146663 P3h2

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, Golgi apparatus, Sarcoplasmic reticulum

Pathology & Biotechi

Disruption phenotypei

Contradictory results have been described and may be due to differences in the methods used for gene disruption (PubMed:24368846, PubMed:25645914). No visible phenotype, but decreased 3-hydroxyproline formation in collagen COL4A1 and COL1A1 (PubMed:25645914). Full embryonic lethality; the vast majority die before 8.5 dpc (PubMed:24368846). The embryos appear normal, but are surrounded by maternal platelet aggregates and blood clots that form at about 6.5 dpc, resulting in embryonic death (PubMed:24368846). Maternal platelet aggregation is triggered by interaction between maternal Gp6 and embryonic type IV collagen that lacks 3-hydroxyproline (PubMed:24368846). Likewise, pregnant females deficient for Gp6 and P3h2 that bear embryos deficient in P3h2 and heterozygous for Gp6 do not produce any live offspring (PubMed:24368846). In contrast, mutant mice deficient in Gp6 and P3h2 are born at the expected Mendelian rate and have no visible phenotype (PubMed:24368846).2 Publications

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 21Sequence analysisAdd BLAST21
ChainiPRO_000024035722 – 703Prolyl 3-hydroxylase 2Add BLAST682

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi444N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi544N-linked (GlcNAc...) asparagineSequence analysis1

Keywords - PTMi

Glycoprotein

Proteomic databases

MaxQBiQ8CG71
PaxDbiQ8CG71
PeptideAtlasiQ8CG71
PRIDEiQ8CG71

PTM databases

iPTMnetiQ8CG71
PhosphoSitePlusiQ8CG71

Expressioni

Tissue specificityi

Detected in kidney (PubMed:25645914). Detected on kidney tubular cells, pancreas acinar cells, Schwann cells of the peripheral nerve in the pinna, and in tunica adventitia, the smooth muscle layer of the aortic wall (at protein level) (PubMed:18487197). Detected in lung, skeletal muscle and kidney (PubMed:18487197). Detected in kidney glomeruli and in prehypertrophic regions of long bone from neonates (PubMed:25645914). In the eye, detected in the epithelial layer of the cornea and at lower levels in the sclera at the posterior end of the eye (PubMed:25645914).2 Publications

Gene expression databases

BgeeiENSMUSG00000038168
CleanExiMM_LEPREL1
GenevisibleiQ8CG71 MM

Interactioni

Protein-protein interaction databases

IntActiQ8CG71, 1 interactor
STRINGi10090.ENSMUSP00000038056

Structurei

3D structure databases

ProteinModelPortaliQ8CG71
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Repeati42 – 75TPR 1Add BLAST34
Repeati144 – 177TPR 2Add BLAST34
Repeati205 – 238TPR 3Add BLAST34
Repeati301 – 334TPR 4Add BLAST34
Domaini552 – 666Fe2OG dioxygenasePROSITE-ProRule annotationAdd BLAST115

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi700 – 703Prevents secretion from ERPROSITE-ProRule annotation4

Sequence similaritiesi

Belongs to the leprecan family.Curated

Keywords - Domaini

Repeat, Signal, TPR repeat

Phylogenomic databases

eggNOGiKOG4459 Eukaryota
ENOG410XPXF LUCA
GeneTreeiENSGT00550000074573
HOGENOMiHOG000231087
HOVERGENiHBG053224
InParanoidiQ8CG71
KOiK22459
OMAiNINPKDE
OrthoDBiEOG091G06WO
PhylomeDBiQ8CG71
TreeFamiTF320837

Family and domain databases

InterProiView protein in InterPro
IPR005123 Oxoglu/Fe-dep_dioxygenase
IPR006620 Pro_4_hyd_alph
IPR011990 TPR-like_helical_dom_sf
PfamiView protein in Pfam
PF13640 2OG-FeII_Oxy_3, 1 hit
SMARTiView protein in SMART
SM00702 P4Hc, 1 hit
SUPFAMiSSF48452 SSF48452, 4 hits
PROSITEiView protein in PROSITE
PS00014 ER_TARGET, 1 hit
PS51471 FE2OG_OXY, 1 hit

Sequencei

Sequence statusi: Complete.

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

Q8CG71-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MRESTWVSLL LLLLLPTPQR GGPQDGRRSP EPEPERGPLQ PFDLLYASGV
60 70 80 90 100
AAYYSGDYER AVRDLEAALS SHRRLRDIRT RCARHCAARR PLAPPGAGPG
110 120 130 140 150
AELPFFRAVL ERARCSRSCQ SQRLGGPASR HRVSEDVRSD FQRRVPYNYL
160 170 180 190 200
QRAYIKLNQL EKAMEAAHTF FMANPEHMEM QQDLEDYKAT ARVEAPLLDR
210 220 230 240 250
EAKPHLESYN AGVKHYEADD FESAIKYFEQ ALREYFNEDM ECRALCEGPQ
260 270 280 290 300
RFEEYEYLGY KGGLYEAIAD HYMQVLVCQH ECVRELATRP GRLSPIENFL
310 320 330 340 350
PLHYDYLQFA YYRVGEYVKA LECAKAYLMF HPDNEDVLDN VDFYESLLDD
360 370 380 390 400
STDPASIEAR EDLTAFVKRH KLEAELIKLA AEGLGFSYAE PNYWISYGGR
410 420 430 440 450
QDENRVPSGV NMDGAEVHGL SMGKKSPPKI GRDLREGGPL LYENITFVYN
460 470 480 490 500
SEQLNGTQRV LLDNVLSQEQ CRELHSVANG IMLVGDGYRG KTSPHTPNEK
510 520 530 540 550
FEGATVLKAL KFGYEGRVPL KSARLFYDIS EKARKIVESY FMLNSTLYFS
560 570 580 590 600
YTHMVCRTAL SGQQDRRNDL SHPIHADNCL LDPEANECWK EPPAYTFRDY
610 620 630 640 650
SALLYMNDDF DGGEFIFTEM DAKTVTASIK PKCGRMISFS SGGENPHGVK
660 670 680 690 700
AVTRGQRCAV ALWFTLDPLY RELERIQADE VIAILDQEQR GKHGLNINPK

DEL
Length:703
Mass (Da):80,154
Last modified:March 1, 2003 - v1
Checksum:i1F42F9B9938573E4
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti497P → A in BAC36342 (PubMed:16141072).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ430350 mRNA Translation: CAD23038.1
AK076434 mRNA Translation: BAC36342.1
CCDSiCCDS28086.1
RefSeqiNP_775555.1, NM_173379.2
UniGeneiMm.326869

Genome annotation databases

EnsembliENSMUST00000039990; ENSMUSP00000038056; ENSMUSG00000038168
GeneIDi210530
KEGGimmu:210530
UCSCiuc007yuy.1 mouse

Similar proteinsi

Entry informationi

Entry nameiP3H2_MOUSE
AccessioniPrimary (citable) accession number: Q8CG71
Secondary accession number(s): Q8C673
Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 27, 2006
Last sequence update: March 1, 2003
Last modified: April 25, 2018
This is version 122 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

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