Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Prolyl 3-hydroxylase 3

Gene

P3h3

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

Functioni

Part of a complex composed of PLOD1, P3H3 and P3H4 that catalyzes hydroxylation of lysine residues in collagen alpha chains and is required for normal assembly and cross-linkling of collagen fibrils (PubMed:27119146). Required for normal hydroxylation of lysine residues in type I collagen chains in skin, bone, tendon, aorta and cornea (PubMed:28115524). Required for normal skin stability via its role in hydroxylation of lysine residues in collagen alpha chains and in collagen fibril assembly (PubMed:27119146, PubMed:28115524). Apparently not required for normal prolyl 3-hydroxylation on collagen chains, possibly because it functions redundantly with other prolyl 3-hydroxylases (PubMed:28115524).2 Publications

Catalytic activityi

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

Cofactori

Protein has several cofactor binding sites:

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi580Iron1
Metal bindingi582Iron1
Metal bindingi652Iron1
Active sitei662By similarity1

GO - Molecular functioni

GO - Biological processi

  • collagen biosynthetic process Source: UniProtKB
  • collagen metabolic process Source: GO_Central
  • negative regulation of cell proliferation Source: MGI
  • peptidyl-lysine 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 3Imported (EC:1.14.11.7)
Alternative name(s):
Leprecan-like protein 21 Publication
Protein BBy similarity
Gene namesi
Name:P3h3Imported
Synonyms:Leprel21 Publication
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 6

Organism-specific databases

MGIiMGI:1315208 P3h3

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

Disruption phenotypei

No visible phenotype at birth. Lysine hydroxylation of skin and bone collagen alpha chains is strongly reduced. In contrast, prolyl 3-hydroxylation is not affected, possibly due to complementation by other family members. Dorsal skin displays impaired packing of collagen fibrils, decreased skin tensile strength, and increased skin fragility. Likewise, mice deficient for both P3h3 and P3h4 display decreased lysine hydroxylation of collagen alpha chains, but normal collagen prolyl 3-hydroxylation.1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 19Sequence analysisAdd BLAST19
ChainiPRO_000024036120 – 732Prolyl 3-hydroxylase 3Add BLAST713

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi327N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi458N-linked (GlcNAc...) asparagineSequence analysis1

Keywords - PTMi

Glycoprotein

Proteomic databases

MaxQBiQ8CG70
PaxDbiQ8CG70
PeptideAtlasiQ8CG70
PRIDEiQ8CG70

PTM databases

PhosphoSitePlusiQ8CG70

Expressioni

Tissue specificityi

Detected in kidney (at protein level).1 Publication

Gene expression databases

BgeeiENSMUSG00000023191
CleanExiMM_LEPREL2
ExpressionAtlasiQ8CG70 baseline and differential
GenevisibleiQ8CG70 MM

Interactioni

Subunit structurei

Identified in a complex with PLOD1 and P3H4.1 Publication

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000023958

Structurei

3D structure databases

ProteinModelPortaliQ8CG70
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Repeati39 – 72TPR 1Add BLAST34
Repeati152 – 185TPR 2Add BLAST34
Repeati214 – 247TPR 3Add BLAST34
Repeati312 – 345TPR 4Add BLAST34
Domaini557 – 671Fe2OG dioxygenasePROSITE-ProRule annotationAdd BLAST115

Coiled coil

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Coiled coili674 – 703Sequence analysisAdd BLAST30

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi729 – 732Prevents secretion from ERPROSITE-ProRule annotation4

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi14 – 39Pro-richAdd BLAST26
Compositional biasi689 – 697Poly-Glu9

Sequence similaritiesi

Belongs to the leprecan family.Curated

Keywords - Domaini

Coiled coil, Repeat, Signal, TPR repeat

Phylogenomic databases

eggNOGiKOG4459 Eukaryota
ENOG410XPXF LUCA
GeneTreeiENSGT00550000074573
HOGENOMiHOG000231087
HOVERGENiHBG053224
InParanoidiQ8CG70
KOiK22460
OMAiAYYQLKK
OrthoDBiEOG091G06WO
PhylomeDBiQ8CG70
TreeFamiTF320837

Family and domain databases

InterProiView protein in InterPro
IPR005123 Oxoglu/Fe-dep_dioxygenase
IPR006620 Pro_4_hyd_alph
PfamiView protein in Pfam
PF13640 2OG-FeII_Oxy_3, 1 hit
SMARTiView protein in SMART
SM00702 P4Hc, 1 hit
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.

Q8CG70-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MLRLLRLLLL LLLPPPGSPE PPEPPGLAQL SPGSPPQAPD LLYADGLRAY
60 70 80 90 100
SAGAWAPAVA LLREALRSRA ALGRARQECG ASCAAEPGAA LPSQLLGAPH
110 120 130 140 150
PVSGPGVWEP LLLRATLRRA ECLTQCAVRR LGPGGAARLR VGSALRDAFR
160 170 180 190 200
RREPYNYLQR AYYQLKKLDL AASAAHTFFV ANPTHLQMRE DMAKYRRMSA
210 220 230 240 250
IRPQSFRDLV TPLYWAAYDT GLELLEQREA ALALPQLEEA LQGSLAHMES
260 270 280 290 300
CRAACEGPEE HQGAEEEGEG SQGGLYEAIA GHWIRVLQCR QHCVADTATR
310 320 330 340 350
PGRSFPVQDF LLSQLRRLHE AYAQVGNMSQ AMENVLSVLL FYPEDEAAKK
360 370 380 390 400
ALNQYQTQLG EPRPDLGPRE DIQRFILRSL GEKRQLYYAM EHLGTSFKDP
410 420 430 440 450
DSWTPEALIP KALRERLRED QEKKPWDHQP PQQKPLAHWK DALLMEGVTL
460 470 480 490 500
TQDAQQLNGS ERAVLDGLLT SAECGVLLQL AKDAAQAGAR SGYRGRRSPH
510 520 530 540 550
SPHERFEGLT VLKAAQLARA GTVGRPGAKL LLEVSERVRT LTQAYFSPER
560 570 580 590 600
PLHLSFTHLV CRSAIEGEQE QRMDLSHPVH ADNCVLDPDT GECWREPPAY
610 620 630 640 650
TYRDYSGLLY LNDDFKGGDL FFTQPNALTV TAQVRPRCGR LVAFSSGGEN
660 670 680 690 700
PHGVWAVTRG RRCALALWHT WAPEHSEQEW TEAKELLQEE EEEEEEEDIL
710 720 730
SRDPSPEPPS HKLQRVQEKA GKPRRVRVRE EL
Length:732
Mass (Da):81,701
Last modified:March 1, 2003 - v1
Checksum:iBD54733B60F1687A
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ441086 mRNA Translation: CAD29580.1
AY463530 mRNA Translation: AAS45239.1
BC003726 mRNA Translation: AAH03726.1
BC016431 mRNA Translation: AAH16431.1
AC002397 Genomic DNA Translation: AAC36012.1
CCDSiCCDS20533.1
RefSeqiNP_038562.2, NM_013534.4
UniGeneiMm.35708
Mm.9002

Genome annotation databases

EnsembliENSMUST00000023958; ENSMUSP00000023958; ENSMUSG00000023191
GeneIDi14789
KEGGimmu:14789
UCSCiuc009dsf.1 mouse

Similar proteinsi

Entry informationi

Entry nameiP3H3_MOUSE
AccessioniPrimary (citable) accession number: Q8CG70
Secondary accession number(s): O88836
Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 27, 2006
Last sequence update: March 1, 2003
Last modified: April 25, 2018
This is version 117 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

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. SIMILARITY comments
    Index of protein domains and families

We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.

Do not show this banner again
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health