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Protein

Phytanoyl-CoA dioxygenase, peroxisomal

Gene

Phyh

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

Functioni

Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.

Catalytic activityi

Phytanoyl-CoA + 2-oxoglutarate + O2 = 2-hydroxyphytanoyl-CoA + succinate + CO2.

Cofactori

Protein has several cofactor binding sites:

Pathwayi: fatty acid metabolism

This protein is involved in the pathway fatty acid metabolism, which is part of Lipid metabolism.
View all proteins of this organism that are known to be involved in the pathway fatty acid metabolism and in Lipid metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei120Alpha-ketoglutarateBy similarity1
Binding sitei157Alpha-ketoglutarateBy similarity1
Metal bindingi175IronBy similarity1
Metal bindingi177IronBy similarity1
Binding sitei193Alpha-ketoglutarateBy similarity1
Metal bindingi264IronBy similarity1
Binding sitei266Alpha-ketoglutarateBy similarity1
Binding sitei275Alpha-ketoglutarateBy similarity1

GO - Molecular functioni

GO - Biological processi

Keywordsi

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

Enzyme and pathway databases

ReactomeiR-MMU-389599 Alpha-oxidation of phytanate
R-MMU-9033241 Peroxisomal protein import
UniPathwayiUPA00199

Names & Taxonomyi

Protein namesi
Recommended name:
Phytanoyl-CoA dioxygenase, peroxisomal (EC:1.14.11.18)
Alternative name(s):
Lupus nephritis-associated peptide 1
Phytanic acid oxidase
Phytanoyl-CoA alpha-hydroxylase
Short name:
PhyH
Gene namesi
Name:Phyh
Synonyms:Ln1, Lnap1, Pahx
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 2

Organism-specific databases

MGIiMGI:891978 Phyh

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

Peroxisome

Pathology & Biotechi

Involvement in diseasei

Defects in Phyh are the cause of lupus nephritis, a severe autoimmune disease. Phyh could be involved in a reaction against the progression of the disease, because its expression is low in the early stage of the disease in the renal cortex of MRL/lpr mice.1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 30PeroxisomeBy similarityAdd BLAST30
ChainiPRO_000002405431 – 338Phytanoyl-CoA dioxygenase, peroxisomalAdd BLAST308

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei59N6-succinyllysineCombined sources1
Modified residuei108N6-succinyllysineCombined sources1
Modified residuei231N6-succinyllysineCombined sources1
Modified residuei252N6-succinyllysineCombined sources1

Proteomic databases

EPDiO35386
PaxDbiO35386
PeptideAtlasiO35386
PRIDEiO35386

PTM databases

iPTMnetiO35386
PhosphoSitePlusiO35386
SwissPalmiO35386

Expressioni

Tissue specificityi

Ubiquitously expressed in all tissues with significant levels detected in the embryonic and neonatal heart and liver. In the adult, significant levels are detected in the liver, kidney, heart, gut, brain and aorta.1 Publication

Gene expression databases

BgeeiENSMUSG00000026664
CleanExiMM_PHYH
ExpressionAtlasiO35386 baseline and differential
GenevisibleiO35386 MM

Interactioni

Subunit structurei

Interacts specifically with the immunophilin FKBP52 and PHYHIP.1 Publication

Protein-protein interaction databases

BioGridi201185, 1 interactor
IntActiO35386, 2 interactors
MINTiO35386
STRINGi10090.ENSMUSP00000027975

Structurei

3D structure databases

ProteinModelPortaliO35386
SMRiO35386
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni175 – 177Alpha-ketoglutarate bindingBy similarity3

Sequence similaritiesi

Belongs to the PhyH family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiENOG410IPBI Eukaryota
COG5285 LUCA
GeneTreeiENSGT00390000001775
HOGENOMiHOG000007341
HOVERGENiHBG000392
InParanoidiO35386
KOiK00477
OMAiKMYHGIQ
OrthoDBiEOG091G0F66
PhylomeDBiO35386
TreeFamiTF313667

Family and domain databases

InterProiView protein in InterPro
IPR008775 Phytyl_CoA_dOase
PfamiView protein in Pfam
PF05721 PhyH, 1 hit

Sequencei

Sequence statusi: Complete.

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

O35386-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MNLTRAGARL QVLLGHLGRP SAPTIVAQPV SGLASPASFQ PEQFQYTLDN
60 70 80 90 100
NVLTLEQRKF YEENGFLVIK NLVSDDDIQR FRAEFERICR EEVKPPGIVI
110 120 130 140 150
MRDVALAKQD YMPSDRMVSK IQDFQEDEEL FRYCLLPEIL KYVECFTGPN
160 170 180 190 200
IMALHGMLIN KPPDVGKKTS RHPLHQDLHY FPFRPSNLIV CAWTAMEHID
210 220 230 240 250
RNNGCLVVLP GTHKGTLKPH DYPKWEGGVN KMYHGIQDYD PNSPRVHLVM
260 270 280 290 300
EKGDTVFFHP LLIHGSGRNK TQGFRKAISC HFGSSDCQCI DVSGTSQENI
310 320 330
AREVVEMAEK KYGFQGVMDF KDTWIFRSRL VKGERINI
Length:338
Mass (Da):38,607
Last modified:January 1, 1998 - v1
Checksum:i9363F4322D59360E
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti2N → K in BAA19003 (PubMed:8954131).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF023463 mRNA Translation: AAB81835.1
D88670 mRNA Translation: BAA19003.1
BC002018 mRNA Translation: AAH02018.1
CCDSiCCDS15660.1
PIRiJC5242
RefSeqiNP_034856.1, NM_010726.2
UniGeneiMm.391704

Genome annotation databases

EnsembliENSMUST00000027975; ENSMUSP00000027975; ENSMUSG00000026664
GeneIDi16922
KEGGimmu:16922
UCSCiuc008ifd.2 mouse

Similar proteinsi

Entry informationi

Entry nameiPAHX_MOUSE
AccessioniPrimary (citable) accession number: O35386
Secondary accession number(s): O08527
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1999
Last sequence update: January 1, 1998
Last modified: May 23, 2018
This is version 147 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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