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
ECTree
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Source Tissue
Source Tissue on EC 1.14.18.6 - 4-hydroxysphinganine ceramide fatty acyl 2-hydroxylase
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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
FA2H is expressed in cultured human keratinocytes and human epidermis, with FA2H expression and fatty acid 2-hydroxylase activity increasing with differentiation
expression in white matter. Time course of expression in brain closely follows the expression of myelin-specific genes, reaching a maximum at 2-3 weeks of age