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
+ 2 ferrocytochrome b5 + + 2 H+ = + 2 ferricytochrome b5 +
Synonyms
FA2H, AtFAH1, FA2H, FAH1, fatty acid 2-hydroxylase, fatty acid 2-hydroxylase 1, fatty acid alpha-hydroxylase, SCS7, Scs7p, scScs7p, sphingolipid alpha-hydroxylase
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Localization
Localization on EC 1.14.18.6 - 4-hydroxysphinganine ceramide fatty acyl 2-hydroxylase
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an integral membrane protein, the Scs7p core is composed of a helical catalytic cap domain that sits atop four transmembrane helices that anchor the enzyme in the endoplasmic reticulum
additional information
protein is predicted to have two hydrophobic domains, each capable of spanning the membrane twice, and has the HX(2-3)(XH)H motifs that are characteristic of membrane-bound fatty acid desaturases
modeling of secondary structural elements of the scScs7p hydroxylase domain placed within a model lipid bilayer, overview
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additional information
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modeling of secondary structural elements of the scScs7p hydroxylase domain placed within a model lipid bilayer, overview
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