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. 1999:466:117-24.

Carnitine biosynthesis. Purification of gamma-butyrobetaine hydroxylase from rat liver

F M Vaz  1 , S van Gool, R Ofman, L IJlst, R J Wanders
Affiliations
  • PMID: 10709635

Carnitine biosynthesis. Purification of gamma-butyrobetaine hydroxylase from rat liver

F M Vaz et al. Adv Exp Med Biol. 1999.

Abstract

gamma-Butyrobetaine hydroxylase catalyse the last step in carnitine biosynthesis, the formation of L-carnitine from gamma-butyrobetaine, a reaction dependent on Fe2+, alpha-ketoglutarate, ascorbate and oxygen. Initial attempts to purify the protein from rat liver showed that gamma-butyrobetaine hydroxylase is unstable. We, therefore, determined the influence of various compounds on the stability of gamma-butyrobetaine hydroxylase at different storage temperatures. The enzyme activity was best conserved by storing the protein at 4 degrees C in the presence of 200 g/l glycerol and 10 mM DTT. We subsequently purified the enzyme from rat liver to apparent homogeneity by liquid chromatography.

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