1.14.11.18: phytanoyl-CoA dioxygenase
This is an abbreviated version!
For detailed information about phytanoyl-CoA dioxygenase, go to the full flat file.
Word Map on EC 1.14.11.18
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1.14.11.18
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peroxisomal
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refsum
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alpha-oxidation
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zellweger
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pigmentosa
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beta-oxidation
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3,7,11,15-tetramethylhexadecanoic
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punctata
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pipecolic
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rhizomelic
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pristanic
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2-hydroxyphytanoyl-coa
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chondrodysplasia
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2-hydroxylation
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anosmia
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3-methyl
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phytol
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3-methyl-branched
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cerebro-hepato-renal
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2-oxoglutarate-dependent
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diagnostics
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medicine
- 1.14.11.18
- peroxisomal
- refsum
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alpha-oxidation
- zellweger
-
pigmentosa
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beta-oxidation
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3,7,11,15-tetramethylhexadecanoic
- punctata
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pipecolic
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rhizomelic
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pristanic
- 2-hydroxyphytanoyl-coa
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chondrodysplasia
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2-hydroxylation
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anosmia
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3-methyl
- phytol
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3-methyl-branched
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cerebro-hepato-renal
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2-oxoglutarate-dependent
- diagnostics
- medicine
Reaction
Synonyms
PAHX, PhyH, PHYHD1, Phytanic acid oxidase, phytanoyl-CoA 2-hydroxylase, Phytanoyl-CoA alpha-hydroxylase, phytanoyl-CoA dioxygenase domain containing protein 1, phytanoyl-CoA hydroxylase
ECTree
1.14.11.18 phytanoyl-CoA dioxygenaseAdvanced search results
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results (Substrates Products
Substrates Products on EC 1.14.11.18 - phytanoyl-CoA dioxygenase
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SUBSTRATE 
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
3,7-dimethyloctan-6-enoyl-CoA + 2-oxoglutarate + O2
3,7-dimethyl-2-hydroxyocta-7-enoyl-CoA + succinate + CO2
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75.2 of the activity with 3-methylhexadecanoyl-CoA
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-
?
3-ethylnonanoyl-CoA + 2-oxoglutarate + O2
3-ethoxy-2-hydroxyhexadecanoyl-CoA + succinate + CO2
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64.7% of the activity with 3-methylhexadecanoyl-CoA
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-
?
3-methyl-5-phenyl-pentanoyl-CoA + 2-oxoglutarate + O2
2-hydroxy-3-methyl-5-phenylpentanoyl-CoA + succinate + CO2
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53% of the activity with 3-methylhexadecanoyl-CoA
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-
?
3-methyldodecanoyl-CoA + 2-oxoglutarate + O2
2-hydroxy-3-methyldodecanoyl-CoA + succinate + CO2
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110% of the activity with 3-methylhexadecanoyl-CoA
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-
?
3-methylheptanoyl-CoA + 2-oxoglutarate + O2
2-hydroxy-3-methylheptanoyl-CoA + succinate + CO2
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16.2% of the activity with 3-methylhexadecanoyl-CoA
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-
?
3-methylhexadecanoyl-CoA + 2-oxoglutarate + O2
2-hydroxy-3-methylhexadecanoyl-CoA + succinate + CO2
3-methylnonanoyl-CoA + 2-oxoglutarate + O2
2-hydroxy-3-methylnonanoyl-CoA + succinate + CO2
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111% of the activity with 3-methylhexadecanoyl-CoA
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-
?
3-methylundecanoyl-CoA + 2-oxoglutarate + O2
2-hydroxy-3-methylundecanoyl-CoA + succinate + CO2
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110% of the activity with 3-methylhexadecanoyl-CoA
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-
?
butanoyl-CoA + 2-oxoglutarate + O2
2-hydroxybutanoyl-CoA + succinate + CO2
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-
-
-
?
decanoyl-CoA + 2-oxoglutarate + O2
2-hydroxydecanoyl-CoA + succinate + CO2
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-
-
-
?
dodecanoyl-CoA + 2-oxoglutarate + O2
2-hydroxydodecanoyl-CoA + succinate + CO2
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-
-
-
?
hexadecanoyl-CoA + 2-oxoglutarate + O2
2-hydroxyhexadecanoyl-CoA + succinate + CO2
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-
-
-
?
hexanoyl-CoA + 2-oxoglutarate + O2
2-hydroxyhexanoyl-CoA + succinate + CO2
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-
-
-
?
octadecanoyl-CoA + 2-oxoglutarate + O2
2-hydroxyoctadecanoyl-CoA + succinate + CO2
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-
-
-
?
octanoyl-CoA + 2-oxoglutarate + O2
2-hydroxyoctanoyl-CoA + succinate + CO2
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-
-
-
?
tetradecanoyl-CoA + 2-oxoglutarate + O2
2-hydroxytetradecanoyl-CoA + succinate + CO2
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-
-
-
?
3-methylhexadecanoyl-CoA + 2-oxoglutarate + O2
2-hydroxy-3-methylhexadecanoyl-CoA + succinate + CO2
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-
-
-
?
3-methylhexadecanoyl-CoA + 2-oxoglutarate + O2
2-hydroxy-3-methylhexadecanoyl-CoA + succinate + CO2
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-
-
-
?
phytanoyl-CoA + 2-oxoglutarate + O2
2-hydroxyphytanoyl-CoA + succinate + CO2
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-
-
-
?
phytanoyl-CoA + 2-oxoglutarate + O2
2-hydroxyphytanoyl-CoA + succinate + CO2
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-
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ir
phytanoyl-CoA + 2-oxoglutarate + O2
2-hydroxyphytanoyl-CoA + succinate + CO2
Refsums disease ia a neurological syndrome characterized by adult-onset retinitis pigmentosa, anosemia, sensory neuropathy and phytanic acidaemia. Many cases are caused by mutations in peroxidomal oxygenase phytanoyl-CoA 2-hydroxylase
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-
?
phytanoyl-CoA + 2-oxoglutarate + O2
2-hydroxyphytanoyl-CoA + succinate + CO2
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3R and 3S epimers are hydroxylated with approximately equal efficiency. Both unprocessed proenzyme and mature form of PAHX are fully active
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-
?
phytanoyl-CoA + 2-oxoglutarate + O2
alpha-hydroxyphytanoyl-CoA + succinate + CO2
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-
-
-
?
phytanoyl-CoA + 2-oxoglutarate + O2
alpha-hydroxyphytanoyl-CoA + succinate + CO2
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no activity with octadecanoyl-CoA, lignoceroyl-CoA, 2-methylhexadecanoyl-CoA and 4,8,12-trimethyltridecanoyl-CoA
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-
?
phytanoyl-CoA + 2-oxoglutarate + O2
alpha-hydroxyphytanoyl-CoA + succinate + CO2
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-
-
-
?
additional information
?
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interaction between PAHX and the A2 domain of the coagulation factor VIII is a part responsible for the low-level expression of factor VIII
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-
?
additional information
?
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Resum disease might be characterized by an accumulation of not only phytanic acid but also ther 3-alkyl-branched fatty acids
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-
?
additional information
?
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no activity with isovaleryl-CoA, 4-phenyl-3-methylbutanoyl-CoA, 3-methylpentanoyl-CoA, hexadecanoyl-CoA, octanoyl-CoA, prostanoyl-CoA or 4-methylnonaoyl-CoA. Chain length of at least seven carbon atoms is a prerequisite for PAHX-dependent hydroxylation. The optimal chain length appears to be 9-12 carbon atoms. The branch at position 3 is a prerequisite for the activity of PAHX
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?
