| General information | | DisProt: | DP00327 | | Name: | Phytanoyl-CoA dioxygenase, peroxisomal | | Synonym(s): | PAHX_HUMAN
EC 1.14.11.18
Phytanoyl-CoA alpha-hydroxylase
PhyH
Phytanic acid oxidase
| | First appeared in release: | Release 3.0 (02/17/2006) | | UniProt: | O14832 | | UniGene: | Hs.498732 | | SwissProt: | PAHX_HUMAN | | TrEMBL: | | | NCBI (GI): | 6093646 | | Source organism: | Homo sapiens (Human) | | Sequence length: | 338 | | Percent disordered: | 14% | | Homologues: | |
| Native sequence |
10 20 30 40 50 60
| | | | | |
MEQLRAAARL QIVLGHLGRP SAGAVVAHPT SGTISSASFH PQQFQYTLDN NVLTLEQRKF - 60
YEENGFLVIK NLVPDADIQR FRNEFEKICR KEVKPLGLTV MRDVTISKSE YAPSEKMITK - 120
VQDFQEDKEL FRYCTLPEIL KYVECFTGPN IMAMHTMLIN KPPDSGKKTS RHPLHQDLHY - 180
FPFRPSDLIV CAWTAMEHIS RNNGCLVVLP GTHKGSLKPH DYPKWEGGVN KMFHGIQDYE - 240
ENKARVHLVM EKGDTVFFHP LLIHGSGQNK TQGFRKAISC HFASADCHYI DVKGTSQENI - 300
EKEVVGIAHK FFGAENSVNL KDIWMFRARL VKGERTNL
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| Functional narrative |
Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
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| Map of ordered and disordered regions |
Note: 'Mouse' over a region to see the start and stop residues. Click on a region to see detailed information.
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| Region 1 | | Type: | Disordered | | Name: | | | Location: | 31 - 42 | | Length: | 12 | | Region sequence: |
SGTISSASFHPQ | | Modification type: | Monomeric
| | PDB: | 2A1X:A | | Structural/functional type: | Relationship to function unknown
| | Functional classes: | Unknown
| | Functional subclasses: | Unknown
| Detection methods:
- X-ray crystallography (291 K; pH: 7.1; PEG 3350 21 %; triammonium citrate 0.3 M)
| References:
- McDonough MA, Kavanagh KL, Butler D, Searls T, Oppermann U, Schofield CJ. "Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease." J Biol Chem. 2005; 280(49): 41101-10. PubMed: 16186124
| Comments:
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| Region 2 | | Type: | Disordered | | Name: | | | Location: | 51 - 52 | | Length: | 2 | | Region sequence: |
NV | | Modification type: | Monomeric
| | PDB: | 2A1X:A | | Structural/functional type: | Relationship to function unknown
| | Functional classes: | Unknown
| | Functional subclasses: | Unknown
| Detection methods:
- X-ray crystallography (291 K; pH: 7.1; PEG 3350 21 %; triammonium citrate 0.3 M)
| References:
- McDonough MA, Kavanagh KL, Butler D, Searls T, Oppermann U, Schofield CJ. "Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease." J Biol Chem. 2005; 280(49): 41101-10. PubMed: 16186124
| Comments:
|
| Region 3 | | Type: | Disordered | | Name: | | | Location: | 165 - 172 | | Length: | 8 | | Region sequence: |
SGKKTSRH | | Modification type: | Monomeric
| | PDB: | 2A1X:A | | Structural/functional type: | Function arises via a disorder to order transition
| | Functional classes: | | | Functional subclasses: | Substrate/ligand binding
| Detection methods:
- X-ray crystallography (291 K; pH: 7.1; PEG 3350 21 %; triammonium citrate 0.3 M)
| References:
- McDonough MA, Kavanagh KL, Butler D, Searls T, Oppermann U, Schofield CJ. "Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease." J Biol Chem. 2005; 280(49): 41101-10. PubMed: 16186124
| Comments:
|
| Region 4 | | Type: | Disordered | | Name: | | | Location: | 223 - 233 | | Length: | 11 | | Region sequence: |
PKWEGGVNKMF | | Modification type: | Monomeric
| | PDB: | 2A1X:A | | Structural/functional type: | Function arises via a disorder to order transition
| | Functional classes: | | | Functional subclasses: | Substrate/ligand binding
| Detection methods:
- X-ray crystallography (291 K; pH: 7.1; PEG 3350 21 %; triammonium citrate 0.3 M)
| References:
- McDonough MA, Kavanagh KL, Butler D, Searls T, Oppermann U, Schofield CJ. "Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease." J Biol Chem. 2005; 280(49): 41101-10. PubMed: 16186124
| Comments:
|
| Region 5 | | Type: | Disordered | | Name: | | | Location: | 303 - 318 | | Length: | 16 | | Region sequence: |
EVVGIAHKFFGAENSV | | Modification type: | Monomeric
| | PDB: | 2A1X:A | | Structural/functional type: | Function arises via a disorder to order transition
| | Functional classes: | | | Functional subclasses: | Substrate/ligand binding
| Detection methods:
- X-ray crystallography (291 K; pH: 7.1; PEG 3350 21 %; triammonium citrate 0.3 M)
| References:
- McDonough MA, Kavanagh KL, Butler D, Searls T, Oppermann U, Schofield CJ. "Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease." J Biol Chem. 2005; 280(49): 41101-10. PubMed: 16186124
| Comments:
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| References |
- Jansen GA, Ofman R, Ferdinandusse S, Ijlst L, Muijsers AO, Skjeldal OH, Stokke O, Jakobs C, Besley GT, Wraith JE, Wanders RJ. "Refsum disease is caused by mutations in the phytanoyl-CoA hydroxylase gene." Nat Genet. 1997; 17(2): 190-3. PubMed: 9326940
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