Gene/Proteome Database (LMPD)
LMPD ID
LMP002178
Gene ID
Species
Mus musculus (Mouse)
Gene Name
phytanoyl-CoA hydroxylase
Gene Symbol
Synonyms
AI256161; AI265699; Lnap1; PAHX
Alternate Names
phytanoyl-CoA dioxygenase, peroxisomal; phytanic acid oxidase; phytanoyl-CoA alpha-hydroxylase; lupus nephritis-associated peptide 1
Chromosome
2
Map Location
2 A1|2
EC Number
1.14.11.18
Proteins
| phytanoyl-CoA dioxygenase, peroxisomal precursor | |
|---|---|
| Refseq ID | NP_034856 |
| Protein GI | 6754564 |
| UniProt ID | O35386 |
| mRNA ID | NM_010726 |
| Length | 338 |
| RefSeq Status | VALIDATED |
| MNLTRAGARLQVLLGHLGRPSAPTIVAQPVSGLASPASFQPEQFQYTLDNNVLTLEQRKFYEENGFLVIKNLVSDDDIQRFRAEFERICREEVKPPGIVIMRDVALAKQDYMPSDRMVSKIQDFQEDEELFRYCLLPEILKYVECFTGPNIMALHGMLINKPPDVGKKTSRHPLHQDLHYFPFRPSNLIVCAWTAMEHIDRNNGCLVVLPGTHKGTLKPHDYPKWEGGVNKMYHGIQDYDPNSPRVHLVMEKGDTVFFHPLLIHGSGRNKTQGFRKAISCHFGSSDCQCIDVSGTSQENIAREVVEMAEKKYGFQGVMDFKDTWIFRSRLVKGERINI | |
| transit_peptide: 1..30 inference: non-experimental evidence, no additional details recorded note: Peroxisome (By similarity); propagated from UniProtKB/Swiss-Prot (O35386.1) calculated_mol_wt: 3138 peptide sequence: MNLTRAGARLQVLLGHLGRPSAPTIVAQPV mat_peptide: 31..338 product: Phytanoyl-CoA dioxygenase, peroxisomal experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (O35386.1) calculated_mol_wt: 35488 peptide sequence: SGLASPASFQPEQFQYTLDNNVLTLEQRKFYEENGFLVIKNLVSDDDIQRFRAEFERICREEVKPPGIVIMRDVALAKQDYMPSDRMVSKIQDFQEDEELFRYCLLPEILKYVECFTGPNIMALHGMLINKPPDVGKKTSRHPLHQDLHYFPFRPSNLIVCAWTAMEHIDRNNGCLVVLPGTHKGTLKPHDYPKWEGGVNKMYHGIQDYDPNSPRVHLVMEKGDTVFFHPLLIHGSGRNKTQGFRKAISCHFGSSDCQCIDVSGTSQENIAREVVEMAEKKYGFQGVMDFKDTWIFRSRLVKGERINI | |
Gene Information
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0005739 | IDA:MGI | C | mitochondrion |
| GO:0005777 | TAS:MGI | C | peroxisome |
| GO:0031418 | IEA:UniProtKB-KW | F | L-ascorbic acid binding |
| GO:0003824 | IDA:MGI | F | catalytic activity |
| GO:0048037 | IEA:Ensembl | F | cofactor binding |
| GO:0046872 | IEA:UniProtKB-KW | F | metal ion binding |
| GO:0048244 | IEA:UniProtKB-EC | F | phytanoyl-CoA dioxygenase activity |
| GO:0001561 | IDA:MGI | 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
REACTOME Pathway Links
Domain Information
InterPro Annotations
| Accession | Description |
|---|---|
| IPR008775 | Phytanoyl-CoA dioxygenase |
UniProt Annotations
Entry Information
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; |
| Disease | Note=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. |
| Function | Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA. |
| Pathway | Lipid metabolism; fatty acid metabolism. |
| Similarity | Belongs to the PhyH family. {ECO:0000305}. |
| Subcellular Location | Peroxisome. |
| Subunit | Interacts specifically with the immunophilin FKBP52 and PHYHIP. {ECO:0000269|PubMed:10686344}. |
| Tissue Specificity | 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. {ECO:0000269|PubMed:10686344}. |
Identical and Related Proteins
Unique RefSeq proteins for LMP002178 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 6754564 | RefSeq | NP_034856 | 338 | phytanoyl-CoA dioxygenase, peroxisomal precursor |
Identical Sequences to LMP002178 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:6754564 | DBBJ | BAE40985.1 | 338 | unnamed protein product [Mus musculus] |
| GI:6754564 | DBBJ | BAE27410.1 | 338 | unnamed protein product [Mus musculus] |
| GI:6754564 | DBBJ | BAE37810.1 | 338 | unnamed protein product [Mus musculus] |
| GI:6754564 | DBBJ | BAE38170.1 | 338 | unnamed protein product [Mus musculus] |
| GI:6754564 | GenBank | AAH02018.1 | 338 | Phytanoyl-CoA hydroxylase [Mus musculus] |
| GI:6754564 | GenBank | EDL07951.1 | 338 | phytanoyl-CoA hydroxylase, isoform CRA_a [Mus musculus] |
Related Sequences to LMP002178 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:6754564 | DBBJ | BAA19003.1 | 338 | LN1 [Mus musculus] |
| GI:6754564 | DBBJ | BAE40505.1 | 338 | unnamed protein product [Mus musculus] |
| GI:6754564 | GenBank | AAF15971.1 | 338 | peroxisomal phytanoyl-CoA hydroxylase [Rattus norvegicus] |
| GI:6754564 | GenBank | AAH86573.1 | 338 | Phytanoyl-CoA 2-hydroxylase [Rattus norvegicus] |
| GI:6754564 | GenBank | EDL78672.1 | 338 | phytanoyl-CoA hydroxylase, isoform CRA_a [Rattus norvegicus] |
| GI:6754564 | RefSeq | NP_446126.1 | 338 | phytanoyl-CoA dioxygenase, peroxisomal precursor [Rattus norvegicus] |