• They are Nε-trimethyllysine hydroxylase, 3-hydroxy-Nε-trimethyllysine aldolase, 4-N-trimethylaminobutyraldehyde dehydrogenase and γ-butyrobetaine hydroxylase. (wikipedia.org)
  • The first enzyme of the L-carnitine biosynthetic pathway is Nε-trimethyllysine hydroxylase, an iron and 2-oxoglutarate (2OG)-dependent oxygenase that also requires ascorbate. (wikipedia.org)
  • The final step of L-carnitine biosynthesis is γ-butyrobetaine hydroxylase, a zinc binding enzyme. (wikipedia.org)
  • Like Nε-trimethyllysine hydroxylase, γ-butyrobetaine hydroxylase is a 2-oxoglutarate and iron(II)-dependent oxygenase. (wikipedia.org)
  • γ-Butyrobetaine hydroxylase catalyses the stereospecific hydroxylation of γ-butyrobetaine to L-carnitine. (wikipedia.org)
  • γ-Butyrobetaine hydroxylase is the most studied enzyme among the four enzymes in the biosynthetic pathway. (wikipedia.org)
  • Recombinant human γ-butyrobetaine hydroxylase has also been produced by Escherichia coli and baculoviruses systems. (wikipedia.org)
  • This gene encodes gamma butyrobetaine hydroxylase which catalyzes the formation of L-carnitine from gamma-butyrobetaine, the last step in the L-carnitine biosynthetic pathway. (nih.gov)
  • An enzyme that catalyzes the HYDROXYLATION of gamma-butyrobetaine to L-CARNITINE . (nih.gov)
  • The carnitine biosynthesis pathway is highly conserved among many eukaryotes and some prokaryotes. (wikipedia.org)
  • Activation and Transportation of Fatty Acids for Metabolism via Carnitine Shuttle Pathway (with Animation) Fraenkel, G. (wikipedia.org)
  • Catalyzes the formation of L-carnitine from gamma-butyrobetaine. (nih.gov)
  • At least four enzymes are involved in the overall biosynthetic pathway. (wikipedia.org)
  • use AMINO ACIDS, BRANCHED-CHAIN 1979, & KETO ACIDS & VALERATES 1973-1979 MH - 3-Hydroxyanthranilate 3,4-Dioxygenase UI - D050561 MN - D8.811.682.690.416.328 MS - An enzyme that catalyzes the conversion of 3-hydroxyanthranilate to 2-amino-3-carboxymuconate semialdehyde. (nih.gov)
  • use DICARBOXYLIC ACIDS 1970-1979 MH - 3-Phosphoshikimate 1-Carboxyvinyltransferase UI - D051229 MN - D8.811.913.225.735 MS - An enzyme of the shikimate pathway of AROMATIC AMINO ACID biosynthesis, it generates 5-enolpyruvylshikimate 3-phosphate and ORTHOPHOSPHATE from PHOSPHOENOLPYRUVATE and SHIKIMATE-3-PHOSPHATE. (nih.gov)