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

Reaction

phytanoyl-CoA
+
2-oxoglutarate
+
O2
=
2-hydroxyphytanoyl-CoA
+
succinate
+
CO2

Synonyms

PAHX, PhyH, Phytanic acid oxidase, phytanoyl-CoA 2-hydroxylase, Phytanoyl-CoA alpha-hydroxylase, phytanoyl-CoA hydroxylase

ECTree

     1 Oxidoreductases
         1.14 Acting on paired donors, with incorporation or reduction of molecular oxygen
             1.14.11 With 2-oxoglutarate as one donor, and incorporation of one atom of oxygen into each donor
                1.14.11.18 phytanoyl-CoA dioxygenase

Natural Substrates Products

Natural Substrates Products on EC 1.14.11.18 - phytanoyl-CoA dioxygenase

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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
phytanoyl-CoA + 2-oxoglutarate + O2
2-hydroxyphytanoyl-CoA + succinate + CO2
show the reaction diagram
Refsum‘s 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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additional information
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