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EC 1.14.18.6 Details
show Brenda entryEC number
1.14.18.6
Accepted name
4-hydroxysphinganine ceramide fatty acyl 2-hydroxylase
Reaction
a phytoceramide + 2 ferrocytochrome b5 + O2 + 2 H+ = a (2′R)-2′-hydroxyphytoceramide + 2 ferricytochrome b5 + H2O
Other name(s)
FA2H (gene name),
SCS7 (gene name)
Systematic name
(4R)-4-hydroxysphinganine ceramide,ferrocytochrome-b5:oxygen oxidoreductase (fatty acyl 2-hydroxylating)
Comment
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.
History
created 2015
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1 Oxidoreductases
1.1 Acting on the CH-OH group of donors
1.14.19.1 stearoyl-CoA 9-desaturase
