UniProt Accession

 PAHX_HUMAN; O14832; A8MTS8; B1ALH5 

Genbank Protein ID

 AF023462; AF112977; AF242386; AF242379; AF242380; AF242381; AF242382; AF242383; AF242384; AF242385; AL138764; BC029512 

Genbank Nucleotide ID

 AAB81834.1; AAD20602.1; AAF74123.1; AAF74123.1; AAF74123.1; AAF74123.1; AAF74123.1; AAF74123.1; AAF74123.1; AAF74123.1; CAI12911.2; AAH29512.1 

Protein Name

 Phytanoyl-CoA dioxygenase, peroxisomal 

Protein Synonyms/Alias

 Phytanic acid oxidase; Phytanoyl-CoA alpha-hydroxylase; PhyH 

Gene Name

 PHYH 

Gene Synonyms/Alias

 PAHX 

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
108	MRDVTISKSEYAPSE	ubiquitination	[1, 2]
310	EVVGIAHKFFGAENS	ubiquitination	[1]

Reference

 [1] A proteome-wide, quantitative survey of in vivo ubiquitylation sites reveals widespread regulatory roles.
 Wagner SA, Beli P, Weinert BT, Nielsen ML, Cox J, Mann M, Choudhary C.
 Mol Cell Proteomics. 2011 Oct;10(10):M111.013284. [PMID: 21890473]
 [2] hCKSAAP_UbSite: improved prediction of human ubiquitination sites by exploiting amino acid pattern and properties.
 Chen Z, Zhou Y, Song J, Zhang Z.
 Biochim Biophys Acta. 2013 Aug;1834(8):1461-7. [PMID: 23603789] 

Functional Description

 Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA. 

Sequence Annotation

 REGION 175 177 Alpha-ketoglutarate binding.
 METAL 175 175 Iron.
 METAL 177 177 Iron.
 METAL 264 264 Iron.
 BINDING 120 120 Alpha-ketoglutarate.
 BINDING 157 157 Alpha-ketoglutarate.
 BINDING 193 193 Alpha-ketoglutarate.
 BINDING 266 266 Alpha-ketoglutarate.
 BINDING 275 275 Alpha-ketoglutarate.  

Keyword

 3D-structure; Alternative splicing; Cataract; Complete proteome; Deafness; Dioxygenase; Disease mutation; Ichthyosis; Iron; Metal-binding; Oxidoreductase; Peroxisome; Peroxisome biogenesis disorder; Polymorphism; Reference proteome; Retinitis pigmentosa; Transit peptide; Vitamin C. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 338 AA 

Protein Sequence

Gene Ontology

 GO:0005739; C:mitochondrion; IEA:Compara.
 GO:0005782; C:peroxisomal matrix; TAS:Reactome.
 GO:0048037; F:cofactor binding; IDA:UniProtKB.
 GO:0009055; F:electron carrier activity; TAS:UniProtKB.
 GO:0031418; F:L-ascorbic acid binding; IEA:UniProtKB-KW.
 GO:0046872; F:metal ion binding; IEA:UniProtKB-KW.
 GO:0016702; F:oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen; IEA:UniProtKB-KW.
 GO:0048244; F:phytanoyl-CoA dioxygenase activity; IDA:UniProtKB.
 GO:0001561; P:fatty acid alpha-oxidation; IDA:UniProtKB.
 GO:0006720; P:isoprenoid metabolic process; IDA:UniProtKB.
 GO:0097089; P:methyl-branched fatty acid metabolic process; IDA:UniProtKB. 

Interpro

 IPR008775; Phytyl_CoA_dOase. 

Pfam

 PF05721; PhyH 

SMART

  

PROSITE

  

PRINTS