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Literature summary for 1.14.11.18 extracted from

  • Foulon, V.; Asselberghs, S.; Geens, W.; Mannaerts, G.P.; Casteels, M.; van Veldhoven, P.P.
    Further studies on the substrate spectrum of phytanoyl-CoA hydroxylase: implications for Refsum disease? (2003), J. Lipid Res., 44, 2349-2355.
    View publication on PubMed
Search for more literature for 1.14.11.18

Activating Compound

Activating Compound Comment Organism Structure
albumin 0.05 mM 3-methylhexadecanoyl-CoA, no hydroxylation in absence of albumin. Hydroxylation of 3-methylnonanoyl-CoA or 3-methyldodecanoyl-CoA is much less dependent on the presence of albumin Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.0408
-
 3-methylhexadecanoyl-CoA
-
 Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Homo sapiens Resum disease might be characterized by an accumulation of not only phytanic acid but also ther 3-alkyl-branched fatty acids ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Purification (Commentary)

Purification (Comment) Organism
-
 Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3,7-dimethyloctan-6-enoyl-CoA + 2-oxoglutarate + O2 75.2 of the activity with 3-methylhexadecanoyl-CoA Homo sapiens 3,7-dimethyl-2-hydroxyocta-7-enoyl-CoA + succinate + CO2
-
 ?
3-ethylnonanoyl-CoA + 2-oxoglutarate + O2 64.7% of the activity with 3-methylhexadecanoyl-CoA Homo sapiens 3-ethoxy-2-hydroxyhexadecanoyl-CoA + succinate + CO2
-
 ?
3-methyl-5-phenyl-pentanoyl-CoA + 2-oxoglutarate + O2 53% of the activity with 3-methylhexadecanoyl-CoA Homo sapiens 2-hydroxy-3-methyl-5-phenylpentanoyl-CoA + succinate + CO2
-
 ?
3-methyldodecanoyl-CoA + 2-oxoglutarate + O2 110% of the activity with 3-methylhexadecanoyl-CoA Homo sapiens 2-hydroxy-3-methyldodecanoyl-CoA + succinate + CO2
-
 ?
3-methylheptanoyl-CoA + 2-oxoglutarate + O2 16.2% of the activity with 3-methylhexadecanoyl-CoA Homo sapiens 2-hydroxy-3-methylheptanoyl-CoA + succinate + CO2
-
 ?
3-methylhexadecanoyl-CoA + 2-oxoglutarate + O2
-
 Homo sapiens 2-hydroxy-3-methylhexadecanoyl-CoA + succinate + CO2
-
 ?
3-methylnonanoyl-CoA + 2-oxoglutarate + O2 111% of the activity with 3-methylhexadecanoyl-CoA Homo sapiens 2-hydroxy-3-methylnonanoyl-CoA + succinate + CO2
-
 ?
3-methylundecanoyl-CoA + 2-oxoglutarate + O2 110% of the activity with 3-methylhexadecanoyl-CoA Homo sapiens 2-hydroxy-3-methylundecanoyl-CoA + succinate + CO2
-
 ?
additional information Resum disease might be characterized by an accumulation of not only phytanic acid but also ther 3-alkyl-branched fatty acids Homo sapiens ?
-
 ?
additional information no activity with isovaleryl-CoA, 4-phenyl-3-methylbutanoyl-CoA, 3-methylpentanoyl-CoA, hexadecanoyl-CoA, octanoyl-CoA, prostanoyl-CoA or 4-methylnonaoyl-CoA. Chain length of at least seven carbon atoms is a prerequisite for PAHX-dependent hydroxylation. The optimal chain length appears to be 9-12 carbon atoms. The branch at position 3 is a prerequisite for the activity of PAHX Homo sapiens ?
-
 ?
phytanoyl-CoA + 2-oxoglutarate + O2
-
 Homo sapiens 2-hydroxyphytanoyl-CoA + succinate + CO2
-
 ?

Synonyms

Synonyms Comment Organism
PAHX
-
 Homo sapiens