Information on EC 1.14.18.6 - 4-hydroxysphinganine ceramide fatty acyl 2-hydroxylase

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The enzyme appears in viruses and cellular organisms

EC NUMBER
COMMENTARY hide
1.14.18.6
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RECOMMENDED NAME
GeneOntology No.
4-hydroxysphinganine ceramide fatty acyl 2-hydroxylase
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REACTION
REACTION DIAGRAM
COMMENTARY hide
ORGANISM
UNIPROT
LITERATURE
a phytoceramide + 2 ferrocytochrome b5 + O2 + 2 H+ = a (2'R)-2'-hydroxyphytoceramide + 2 ferricytochrome b5 + H2O
show the reaction diagram
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PATHWAY
BRENDA Link
KEGG Link
MetaCyc Link
fatty acid alpha-oxidation III
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sphingolipid biosynthesis (yeast)
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SYSTEMATIC NAME
IUBMB Comments
(4R)-4-hydroxysphinganine ceramide,ferrocytochrome-b5:oxygen oxidoreductase (fatty acyl 2-hydroxylating)
The enzyme, characterized from yeast and mammals, catalyses the hydroxylation of carbon 2 of long- or very-long-chain fatty acids attached to (4R)-4-hydroxysphinganine during de novo ceramide synthesis. The enzymes from yeast and from mammals contain an N-terminal cytochrome b5 domain that acts as the direct electron donor to the desaturase active site. The newly introduced 2-hydroxyl group has R-configuration. cf. EC 1.14.18.7, dihydroceramide fatty acyl 2-hydroxylase.
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
physiological function
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
monohexosylceramide + 2 ferrocytochrome b5 + O2 + 2 H+
2'-hydroxymonohexosylceramide + 2 ferricytochrome b5 + H2O
show the reaction diagram
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?
palmitic acid + 2 ferrocytochrome b5 + O2 + 2 H+
(R)-2-hydroxypalmitic acid + 2 ferricytochrome b5 + H2O
show the reaction diagram
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FA2H is stereospecific for the production of (R)-enantiomers
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?
additional information
?
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
cytochrome b5
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
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FA2H is expressed in cultured human keratinocytes and human epidermis, with FA2H expression and fatty acid 2-hydroxylase activity increasing with differentiation
Manually annotated by BRENDA team
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FA2H is expressed in cultured human keratinocytes and human epidermis, with FA2H expression and fatty acid 2-hydroxylase activity increasing with differentiation
Manually annotated by BRENDA team
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Manually annotated by BRENDA team
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
SUBUNITS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
?
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x * 71800, calculated, x * 70000, SDS-PAGE
Cloned/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
expression in CHO cell
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expression in CHO cells
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expression in HCT116 cells
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EXPRESSION
ORGANISM
UNIPROT
LITERATURE
differentiation-dependent up-regulation of ceramide synthesis and fatty acid elongation is accompanied by up-regulation of FA2H
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treatment of D6P2T Schwannoma cells with dibutyryl-cAMP induces exit from the cell cycle with concomitant upregulation of FA2H
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ENGINEERING
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
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FA2H lacking the N-terminal cytochrome b5 domain has little activity
APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
medicine