1.14.18.6: 4-hydroxysphinganine ceramide fatty acyl 2-hydroxylase
This is an abbreviated version!
For detailed information about 4-hydroxysphinganine ceramide fatty acyl 2-hydroxylase, go to the full flat file.
Word Map on EC 1.14.18.6
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1.14.18.6
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paraplegia
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sphingolipids
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2-hydroxylation
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leukodystrophy
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nbias
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atp13a2
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dystonia
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pla2g6
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paraparesis
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galactosylceramide
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sulfatide
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alpha-hydroxylation
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paucimobilis
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hydroxylase-associated
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medicine
- 1.14.18.6
- paraplegia
- sphingolipids
-
2-hydroxylation
- leukodystrophy
- nbias
-
atp13a2
- dystonia
- pla2g6
- paraparesis
- galactosylceramide
- sulfatide
-
alpha-hydroxylation
- paucimobilis
-
hydroxylase-associated
- medicine
Reaction
Synonyms
FA2H, AtFAH1, FA2H, FAH1, fatty acid 2-hydroxylase, fatty acid 2-hydroxylase 1, fatty acid alpha-hydroxylase, SCS7, Scs7p, scScs7p, sphingolipid alpha-hydroxylase
ECTree
1.14.18.6 4-hydroxysphinganine ceramide fatty acyl 2-hydroxylaseAdvanced search results
results (
results (Substrates Products
Substrates Products on EC 1.14.18.6 - 4-hydroxysphinganine ceramide fatty acyl 2-hydroxylase
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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
a phytoceramide + 2 ferrocytochrome b5 + O2 + 2 H+
a (2'R)-2'-hydroxyphytoceramide + 2 ferricytochrome b5 + H2O
monohexosylceramide + 2 ferrocytochrome b5 + O2 + 2 H+
2'-hydroxymonohexosylceramide + 2 ferricytochrome b5 + H2O
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?
palmitic acid + 2 ferrocytochrome b5 + O2 + 2 H+
(R)-2-hydroxypalmitic acid + 2 ferricytochrome b5 + H2O
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FA2H is stereospecific for the production of (R)-enantiomers
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?
phytoceramide + ferrocytochrome b5 + O2 + H+
(2'R)-2'-hydroxyphytoceramide + ferricytochrome b5 + H2O
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-
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?
a phytoceramide + 2 ferrocytochrome b5 + O2 + 2 H+
a (2'R)-2'-hydroxyphytoceramide + 2 ferricytochrome b5 + H2O
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-
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?
a phytoceramide + 2 ferrocytochrome b5 + O2 + 2 H+
a (2'R)-2'-hydroxyphytoceramide + 2 ferricytochrome b5 + H2O
a phytoceramide, containing a 26-carbon very-long-chain fatty acid (VLCFA) moiety, is used as the model substrate
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?
additional information
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2-hydroxylation of fatty acid by FA2H occurs prior to generation of ceramides/glucosylceramides. FA2H expression and 2-hydroxy-free fatty acid production increase early in keratinocyte differentiation, production of 2-hydroxyceramides/2-hydroxyglucosylceramides with longer chain amide-linked fatty acids (more than C24) increases later
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?
additional information
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2-hydroxylation of fatty acid by FA2H occurs prior to generation of ceramides/glucosylceramides. FA2H expression and 2-hydroxy-free fatty acid production increase early in keratinocyte differentiation, production of 2-hydroxyceramides/2-hydroxyglucosylceramides with longer chain amide-linked fatty acids (more than C24) increases later
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?
additional information
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initiates fatty acid alpha-oxidation and is also responsible for the biosynthesis of 2-hydroxy fatty acid-containing sphingolipids in mammalian cells
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?
additional information
?
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initiates fatty acid alpha-oxidation and is also responsible for the biosynthesis of 2-hydroxy fatty acid-containing sphingolipids in mammalian cells
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?
additional information
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FA2H is stereospecific for the production of (R)-enantiomers
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?
additional information
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molecular dynamics simulations, allows modeling of a ceramide substrate below the dimetal center of scScs7p
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?
additional information
?
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molecular dynamics simulations, allows modeling of a ceramide substrate below the dimetal center of scScs7p
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?
