Gene/Proteome Database (LMPD)

LMPD ID
LMP012861
Gene ID
114209
Species
Rattus norvegicus (Rat)
Gene Name
phytanoyl-CoA 2-hydroxylase
Gene Symbol
Phyh
Alternate Names
phytanoyl-CoA dioxygenase, peroxisomal; phytanic acid oxidase; phytanoyl-CoA alpha-hydroxylase; phytanoyl-CoA hydroxylase (Refsum disease);
Chromosome
17
Map Location
17q12.3
EC Number
1.14.11.18
Summary
catalyzes the conversion of phytanoyl-CoA to 2-hydroxyphytanoyl-CoA; plays a role in phytanic acid alpha-oxidation [RGD, Feb 2006]
Orthologs
View orthologs and multiple alignments for Phyh

Proteins

phytanoyl-CoA dioxygenase, peroxisomal precursor
Refseq ID NP_446126
Protein GI 16758486
UniProt ID P57093
mRNA ID NM_053674
Length 338
MDYTRAGARLQVLLGHLGRPSALQIVAHPVSGPASPANFCPEQFQYTLDNNVLSLEQRKFYEENGFLVIKNLVSDDDIQRFRAEFERICRKEVKPPGMTVMKDVAIAKQGYAPSERVVTKIQDFQQNEELFRYCALPQIVKYVECFTGPNIMAMHTMLINKPPDSGKKTSRHPLHQDLHFFPFRPSNLIVCAWTAMEHIDRNNGCLVVLPGTHKGPLKPHDYPKWEGGVNKMYHGIQDYDPDSPRVHLVMEKGDTVFFHPLLIHGSGRNRTQGFRKAISCHYGSSDCKYISVKGTSQENIAREVIEIAEKRYGVQGALDFEDTWKFRCRLVKGERINL
transit_peptide: 1..30 experiment: experimental evidence, no additional details recorded note: Peroxisome; propagated from UniProtKB/Swiss-Prot (P57093.2) calculated_mol_wt: 3241 peptide sequence: MDYTRAGARLQVLLGHLGRPSALQIVAHPV mat_peptide: 31..338 product: Phytanoyl-CoA dioxygenase, peroxisomal experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (P57093.2) calculated_mol_wt: 35366 peptide sequence: SGPASPANFCPEQFQYTLDNNVLSLEQRKFYEENGFLVIKNLVSDDDIQRFRAEFERICRKEVKPPGMTVMKDVAIAKQGYAPSERVVTKIQDFQQNEELFRYCALPQIVKYVECFTGPNIMAMHTMLINKPPDSGKKTSRHPLHQDLHFFPFRPSNLIVCAWTAMEHIDRNNGCLVVLPGTHKGPLKPHDYPKWEGGVNKMYHGIQDYDPDSPRVHLVMEKGDTVFFHPLLIHGSGRNRTQGFRKAISCHYGSSDCKYISVKGTSQENIAREVIEIAEKRYGVQGALDFEDTWKFRCRLVKGERINL

Gene Information

Entrez Gene ID
114209
Gene Name
phytanoyl-CoA 2-hydroxylase
Gene Symbol
Phyh
Species
Rattus norvegicus

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005739 IEA:Ensembl C mitochondrion
GO:0005777 IDA:HGNC C peroxisome
GO:0031418 IEA:UniProtKB-KW F L-ascorbic acid binding
GO:0048037 IEA:Ensembl F cofactor binding
GO:0046872 IEA:UniProtKB-KW F metal ion binding
GO:0048244 IDA:RGD F phytanoyl-CoA dioxygenase activity
GO:0001561 TAS:RGD P fatty acid alpha-oxidation
GO:0006720 IEA:Ensembl P isoprenoid metabolic process
GO:0097089 IEA:Ensembl P methyl-branched fatty acid metabolic process

KEGG Pathway Links

KEGG Pathway ID Description
ko04146 Peroxisome
rno04146 Peroxisome

BIOCYC Pathway Links

BIOCYC Pathway ID Description
PWY66-387 fatty acid alpha-oxidation II

REACTOME Pathway Links

REACTOME Pathway ID Description
5954179 Alpha-oxidation of phytanate
5953250 Metabolism
5953289 Metabolism of lipids and lipoproteins
5953470 Peroxisomal lipid metabolism

Domain Information

InterPro Annotations

Accession Description
IPR008775 Phytanoyl-CoA dioxygenase

UniProt Annotations

Entry Information

Gene Name
phytanoyl-CoA 2-hydroxylase
Protein Entry
PAHX_RAT
UniProt ID
P57093
Species
Rat

Comments

Comment Type Description
Catalytic Activity Phytanoyl-CoA + 2-oxoglutarate + O(2) = 2- hydroxyphytanoyl-CoA + succinate + CO(2).
Cofactor Name=Fe cation; Xref=ChEBI:CHEBI:24875;
Cofactor Name=L-ascorbate; Xref=ChEBI:CHEBI:38290;
Function Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
Pathway Lipid metabolism; fatty acid metabolism.
Similarity Belongs to the PhyH family
Subcellular Location Peroxisome.
Subunit Interacts specifically with the immunophilin FKBP52 and PHYHIP

Identical and Related Proteins

Unique RefSeq proteins for LMP012861 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
16758486 RefSeq NP_446126 338 phytanoyl-CoA dioxygenase, peroxisomal precursor

Identical Sequences to LMP012861 proteins

Reference Database Accession Length Protein Name
GI:16758486 GenBank AAH86573.1 338 Phytanoyl-CoA 2-hydroxylase [Rattus norvegicus]
GI:16758486 GenBank EDL78672.1 338 phytanoyl-CoA hydroxylase, isoform CRA_a [Rattus norvegicus]
GI:16758486 GenBank EDL78673.1 338 phytanoyl-CoA hydroxylase, isoform CRA_a [Rattus norvegicus]
GI:16758486 SwissProt P57093.2 338 RecName: Full=Phytanoyl-CoA dioxygenase, peroxisomal; AltName: Full=Phytanic acid oxidase; AltName: Full=Phytanoyl-CoA alpha-hydroxylase; Short=PhyH; Flags: Precursor [Rattus norvegicus]

Related Sequences to LMP012861 proteins

Reference Database Accession Length Protein Name
GI:16758486 GenBank AAF15971.1 338 peroxisomal phytanoyl-CoA hydroxylase [Rattus norvegicus]
GI:16758486 GenBank AAH86573.1 338 Phytanoyl-CoA 2-hydroxylase [Rattus norvegicus]
GI:16758486 GenBank EDL78672.1 338 phytanoyl-CoA hydroxylase, isoform CRA_a [Rattus norvegicus]