Purification and characterization of the rat liver gamma-butyrobetaine hydroxylase

  title={Purification and characterization of the rat liver gamma-butyrobetaine hydroxylase},
  author={S. Galland and F. Le Borgne and D. Guyonnet and P. Clouet and J. Demarquoy},
  journal={Molecular and Cellular Biochemistry},
  • S. Galland, F. Le Borgne, +2 authors J. Demarquoy
  • Published 2004
  • Chemistry, Medicine
  • Molecular and Cellular Biochemistry
  • The biosynthesis of carnitine from lysine and methionine involves five enzymatic reactions. γ-butyrobetaine hydroxylase (BBH; EC is the last enzyme of this pathway. It catalyzes the reaction of hydroxylation of γ-butyrobetaine to carnitine. This enzyme had never been purified to homogeneity from rat tissue. This paper describes the purification and characterization of the rat liver BBH. This protein has been purified some 413 fold by ion exchange, affinity and gel-filtration… CONTINUE READING
    24 Citations
    Crystal structure of human gamma-butyrobetaine hydroxylase.
    • 28
    Structural and mechanistic studies on γ-butyrobetaine hydroxylase.
    • 54
    Analysis of carnitine and acylcaritines in biological fluids and application to a clinical study
    • 2
    • PDF


    Purification and properties of calf liver γ-butyrobetaine hydroxylase
    • 30
    Cofactor requirements of gamma-butyrobetaine hydroxylase from rat liver.
    • 89
    gamma-Butyrobetaine hydroxylase. Structural characterization of the Pseudomonas enzyme.
    • 15
    Carnitine biosynthesis. Purification of 4-N'-trimethylaminobutyraldehyde dehydrogenase from beef liver.
    • 40
    γ-Butyrobetaine hydroxylase in human kidney
    • 21
    Multiple forms of gamma-butyrobetaine hydroxylase (EC
    • 22
    Tissue distribution of carnitine biosynthetic enzymes in man.
    • 197
    Carnitine biosynthesis. beta-Hydroxylation of trimethyllysine by an alpha-ketoglutarate-dependent mitochondrial dioxygenase.
    • 156