Names & Taxonomy
- Uniprot ID:
- O14832
- Entry Name:
- PAHX_HUMAN
- Status:
- reviewed
- Protein Names:
- Phytanoyl-CoA dioxygenase, peroxisomal (EC 1.14.11.18) (Phytanic acid oxidase) (Phytanoyl-CoA alpha-hydroxylase) (PhyH)
- Gene Names:
- PHYH PAHX
- Gene Names Primary:
- PHYH
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 338
- Sequence:
- MEQLRAAARLQIVLGHLGRPSAGAVVAHPTSGTISSASFHPQQFQYTLDNNVLTLEQRKFYEENGFLVIKNLVPDADIQRFRNEFEKICRKEVKPLGLTVMRDVTISKSEYAPSEKMITKVQDFQEDKELFRYCTLPEILKYVECFTGPNIMAMHTMLINKPPDSGKKTSRHPLHQDLHYFPFRPSDLIVCAWTAMEHISRNNGCLVVLPGTHKGSLKPHDYPKWEGGVNKMFHGIQDYEENKARVHLVMEKGDTVFFHPLLIHGSGQNKTQGFRKAISCHFASADCHYIDVKGTSQENIEKEVVGIAHKFFGAENSVNLKDIWMFRARLVKGERTNL
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Peroxisome.
Function
- Function:
- Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
- Pathway:
- Lipid metabolism; fatty acid metabolism.
- Catalytic Activity:
- Phytanoyl-CoA + 2-oxoglutarate + O(2) = 2-hydroxyphytanoyl-CoA + succinate + CO(2).
- Cofactor:
- COFACTOR: Name=Fe cation; Xref=ChEBI:CHEBI:24875;
- Cross Reference Drug Bank:
- DB00025 DB00126
- Gene Ontology Go:
- mitochondrion
peroxisomal matrix
peroxisome
cofactor binding
L-ascorbic acid binding
metal ion binding
phytanoyl-CoA dioxygenase activity
cellular lipid metabolic process
fatty acid alpha-oxidation
isoprenoid metabolic process
methyl-branched fatty acid metabolic process
small molecule metabolic process - Gene Ontology Biological Process:
- cellular lipid metabolic process
fatty acid alpha-oxidation
isoprenoid metabolic process
methyl-branched fatty acid metabolic process
small molecule metabolic process - Gene Ontology Molecular Function:
- cofactor binding
L-ascorbic acid binding
metal ion binding
phytanoyl-CoA dioxygenase activity - Gene Ontology Cellular Component:
- mitochondrion
peroxisomal matrix
peroxisome - Keywords:
- 3D-structure
Alternative splicing
Cataract
Complete proteome
Deafness
Dioxygenase
Disease mutation
Ichthyosis
Iron
Metal-binding
Oxidoreductase
Peroxisome
Peroxisome biogenesis disorder
Phosphoprotein
Polymorphism
Reference proteome
Retinitis pigmentosa
Transit peptide
Vitamin C - Interacts With:
- Q8IZU0