1.14.18.6: 4-hydroxysphinganine ceramide fatty acyl 2-hydroxylase

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For detailed information about 4-hydroxysphinganine ceramide fatty acyl 2-hydroxylase, go to the full flat file.

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 Oxidoreductases

         1.14 Acting on paired donors, with incorporation or reduction of molecular oxygen

             1.14.18 With another compound as one donor, and incorporation of one atom of oxygen into the other donor

                1.14.18.6 4-hydroxysphinganine ceramide fatty acyl 2-hydroxylase

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Results	in table
1	Activating Compound
3	AA Sequence
2	Application
5	Cloned(Commentary)
6	Cofactor
2	Crystallization (Commentary)
210	Disease/ Diagnostics
5	Expression
16	General Information
6	Localization
2	Metals/Ions
2	Molecular Weight [Da]
1	Natural Substrates/ Products (Substrates)
19	Organism
2	Pathway
18	Protein Variants
2	Purification (Commentary)
1	Reaction
19	Reference
14	Source Tissue
9	Substrates and Products (Substrate)
2	Subunits
24	Synonyms
1	Systematic Name

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

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APPLICATION

ORGANISM

UNIPROT

COMMENTARY

LITERATURE

medicine

2 entries

medicine

Homo sapiens

-

in blood and fibroblasts from patients harboring a deleterious FA2H mutatation, hydroxylated fatty acid sphingomyelin is present in normal amounts in patient lymphocytes, but decreases to a different extent in fibroblasts and erythrocytes

734599

medicine

Homo sapiens

Q7L5A8

silencing of sphingolipid fatty acyl 2-hydroxylase Fa2h turns the cells resistant to synthetic antitumor drug PM02734, i.e. elisidepsin. Overexpression of Fa2H leads to an increased sensitivity to PM02734. Exogenous addition of the 2-hydroxylated fatty acid 2-hydroxy palmitic acid to different human cell lines increases their sensitivity to the cytotoxic compound

733604