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Protein

4-hydroxyphenylpyruvate dioxygenase

Gene

Hpd

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Key enzyme in the degradation of tyrosine.By similarity

Catalytic activityi

4-hydroxyphenylpyruvate + O2 = homogentisate + CO2.

Cofactori

Fe cationBy similarityNote: Binds 1 Fe cation per subunit.By similarity

Pathwayi: L-phenylalanine degradation

This protein is involved in step 3 of the subpathway that synthesizes acetoacetate and fumarate from L-phenylalanine.
Proteins known to be involved in the 6 steps of the subpathway in this organism are:
  1. Phenylalanine-4-hydroxylase (Pah)
  2. Tyrosine aminotransferase (Tat)
  3. 4-hydroxyphenylpyruvate dioxygenase (Hpd)
  4. Homogentisate 1,2-dioxygenase (Hgd)
  5. Maleylacetoacetate isomerase (Gstz1)
  6. Fumarylacetoacetase (Fah)
This subpathway is part of the pathway L-phenylalanine degradation, which is itself part of Amino-acid degradation.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes acetoacetate and fumarate from L-phenylalanine, the pathway L-phenylalanine degradation and in Amino-acid degradation.

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi183 – 1831IronBy similarity
Metal bindingi266 – 2661IronBy similarity
Metal bindingi349 – 3491IronBy similarity

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Dioxygenase, Oxidoreductase

Keywords - Biological processi

Phenylalanine catabolism, Tyrosine catabolism

Keywords - Ligandi

Iron, Metal-binding

Enzyme and pathway databases

ReactomeiR-MMU-71182. Phenylalanine and tyrosine catabolism.
SABIO-RKP49429.
UniPathwayiUPA00139; UER00362.

Names & Taxonomyi

Protein namesi
Recommended name:
4-hydroxyphenylpyruvate dioxygenase (EC:1.13.11.27)
Alternative name(s):
4-hydroxyphenylpyruvic acid oxidase
Short name:
4HPPD
Short name:
HPD
Short name:
HPPDase
F Alloantigen
Short name:
F protein
Gene namesi
Name:Hpd
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 5

Organism-specific databases

MGIiMGI:96213. Hpd.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm, Membrane

Pathology & Biotechi

Involvement in diseasei

Defects in Hpd are the cause of tyrosinemia type III.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Initiator methionineiRemovedBy similarity
Chaini2 – 3933924-hydroxyphenylpyruvate dioxygenasePRO_0000088389Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei2 – 21N-acetylthreonineBy similarity
Modified residuei132 – 1321N6-succinyllysineCombined sources
Modified residuei211 – 2111PhosphoserineCombined sources
Modified residuei226 – 2261PhosphoserineCombined sources
Modified residuei250 – 2501PhosphoserineCombined sources

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

MaxQBiP49429.
PaxDbiP49429.
PeptideAtlasiP49429.
PRIDEiP49429.

PTM databases

iPTMnetiP49429.
PhosphoSiteiP49429.
SwissPalmiP49429.

Expressioni

Gene expression databases

BgeeiENSMUSG00000029445.
CleanExiMM_HPD.
ExpressionAtlasiP49429. baseline and differential.
GenevisibleiP49429. MM.

Interactioni

Subunit structurei

Homodimer.By similarity

Protein-protein interaction databases

IntActiP49429. 3 interactions.
MINTiMINT-1869831.
STRINGi10090.ENSMUSP00000031398.

Structurei

3D structure databases

ProteinModelPortaliP49429.
SMRiP49429. Positions 10-384.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the 4HPPD family.Curated

Phylogenomic databases

eggNOGiKOG0638. Eukaryota.
COG3185. LUCA.
GeneTreeiENSGT00530000063474.
HOGENOMiHOG000188687.
HOVERGENiHBG005987.
InParanoidiP49429.
KOiK00457.
OMAiAKQAAHY.
OrthoDBiEOG091G07Y5.
PhylomeDBiP49429.
TreeFamiTF300622.

Family and domain databases

Gene3Di3.10.180.10. 2 hits.
InterProiIPR005956. 4OHPhenylPyrv_dOase.
IPR029068. Glyas_Bleomycin-R_OHBP_Dase.
IPR004360. Glyas_Fos-R_dOase_dom.
[Graphical view]
PANTHERiPTHR11959. PTHR11959. 1 hit.
PfamiPF00903. Glyoxalase. 2 hits.
[Graphical view]
PIRSFiPIRSF009283. HPP_dOase. 1 hit.
SUPFAMiSSF54593. SSF54593. 1 hit.
TIGRFAMsiTIGR01263. 4HPPD. 1 hit.

Sequencei

Sequence statusi: Complete.

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

P49429-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MTTYNNKGPK PERGRFLHFH SVTFWVGNAK QAASFYCNKM GFEPLAYRGL
60 70 80 90 100
ETGSREVVSH VIKQGKIVFV LCSALNPWNK EMGDHLVKHG DGVKDIAFEV
110 120 130 140 150
EDCDHIVQKA RERGAKIVRE PWVEQDKFGK VKFAVLQTYG DTTHTLVEKI
160 170 180 190 200
NYTGRFLPGF EAPTYKDTLL PKLPRCNLEI IDHIVGNQPD QEMQSASEWY
210 220 230 240 250
LKNLQFHRFW SVDDTQVHTE YSSLRSIVVT NYEESIKMPI NEPAPGRKKS
260 270 280 290 300
QIQEYVDYNG GAGVQHIALK TEDIITAIRH LRERGTEFLA APSSYYKLLR
310 320 330 340 350
ENLKSAKIQV KESMDVLEEL HILVDYDEKG YLLQIFTKPM QDRPTLFLEV
360 370 380 390
IQRHNHQGFG AGNFNSLFKA FEEEQALRGN LTDLEPNGVR SGM
Length:393
Mass (Da):45,054
Last modified:January 23, 2007 - v3
Checksum:i70B42A4E4744744A
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti2 – 65TTYNN → VDYWD in CAA42111 (PubMed:1709870).Curated
Sequence conflicti64 – 641Q → R in BAA06267 (PubMed:7774914).Curated
Sequence conflicti120 – 1212EP → DA in CAA42111 (PubMed:1709870).Curated
Sequence conflicti206 – 2061F → S in BAE28861 (PubMed:16141072).Curated

Polymorphismi

There are two alleles (F1 and F2), F2 has Asp-104 and F1 has Asn-104. Mice are completely tolerant to the self form of the protein, but make a good antibody response to immunization with the non-self form.

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti104 – 1041D → N in allele F1. 1 Publication

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D29987 mRNA. Translation: BAA06267.1.
AK149416 mRNA. Translation: BAE28861.1.
BC013343 mRNA. Translation: AAH13343.1.
X59530 mRNA. Translation: CAA42111.1.
CCDSiCCDS39265.1.
PIRiA60236.
RefSeqiNP_032303.1. NM_008277.2.
UniGeneiMm.439709.

Genome annotation databases

EnsembliENSMUST00000031398; ENSMUSP00000031398; ENSMUSG00000029445.
GeneIDi15445.
KEGGimmu:15445.
UCSCiuc008znj.1. mouse.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D29987 mRNA. Translation: BAA06267.1.
AK149416 mRNA. Translation: BAE28861.1.
BC013343 mRNA. Translation: AAH13343.1.
X59530 mRNA. Translation: CAA42111.1.
CCDSiCCDS39265.1.
PIRiA60236.
RefSeqiNP_032303.1. NM_008277.2.
UniGeneiMm.439709.

3D structure databases

ProteinModelPortaliP49429.
SMRiP49429. Positions 10-384.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiP49429. 3 interactions.
MINTiMINT-1869831.
STRINGi10090.ENSMUSP00000031398.

PTM databases

iPTMnetiP49429.
PhosphoSiteiP49429.
SwissPalmiP49429.

Proteomic databases

MaxQBiP49429.
PaxDbiP49429.
PeptideAtlasiP49429.
PRIDEiP49429.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000031398; ENSMUSP00000031398; ENSMUSG00000029445.
GeneIDi15445.
KEGGimmu:15445.
UCSCiuc008znj.1. mouse.

Organism-specific databases

CTDi3242.
MGIiMGI:96213. Hpd.

Phylogenomic databases

eggNOGiKOG0638. Eukaryota.
COG3185. LUCA.
GeneTreeiENSGT00530000063474.
HOGENOMiHOG000188687.
HOVERGENiHBG005987.
InParanoidiP49429.
KOiK00457.
OMAiAKQAAHY.
OrthoDBiEOG091G07Y5.
PhylomeDBiP49429.
TreeFamiTF300622.

Enzyme and pathway databases

UniPathwayiUPA00139; UER00362.
ReactomeiR-MMU-71182. Phenylalanine and tyrosine catabolism.
SABIO-RKP49429.

Miscellaneous databases

PROiP49429.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000029445.
CleanExiMM_HPD.
ExpressionAtlasiP49429. baseline and differential.
GenevisibleiP49429. MM.

Family and domain databases

Gene3Di3.10.180.10. 2 hits.
InterProiIPR005956. 4OHPhenylPyrv_dOase.
IPR029068. Glyas_Bleomycin-R_OHBP_Dase.
IPR004360. Glyas_Fos-R_dOase_dom.
[Graphical view]
PANTHERiPTHR11959. PTHR11959. 1 hit.
PfamiPF00903. Glyoxalase. 2 hits.
[Graphical view]
PIRSFiPIRSF009283. HPP_dOase. 1 hit.
SUPFAMiSSF54593. SSF54593. 1 hit.
TIGRFAMsiTIGR01263. 4HPPD. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiHPPD_MOUSE
AccessioniPrimary (citable) accession number: P49429
Secondary accession number(s): P97322, Q3UEQ0, Q91WV9
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 1, 1996
Last sequence update: January 23, 2007
Last modified: September 7, 2016
This is version 138 of the entry and version 3 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. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. 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.