Dioxygenases of carnitine biosynthesis: 6-N-trimethyllysine and γ-butyrobetaine hydroxylases

Frédéric M. Vaz, Naomi Van Vlies

Research output: Chapter in Book/Report/Conference proceedingChapterAcademicpeer-review

2 Citations (Scopus)

Abstract

This chapter describes the state of knowledge of the two 2-oxoglutarate-dependent dioxygenases of carnitine biosynthesis: 6-N-trimethyllysine hydroxylase and γ-butyrobetaine hydroxylase. Both enzymes have been extensively investigated as carnitine plays an important role in fatty acid metabolism in animals and some other life forms. Carnitine metabolism is introduced followed by a comprehensive review of the properties of the two carnitine biosynthesis dioxygenases including their purification, kinetic and biophysical characterization, regulation and roles in metabolism.

Original languageEnglish
Title of host publication2-Oxoglutarate-Dependent Oxygenases
EditorsRobert P. Hausinger, Christopher J. Schofield
PublisherRoyal Society of Chemistry
Pages324-337
Number of pages14
Edition3
DOIs
Publication statusPublished - 2015

Publication series

NameRSC Metallobiology
Number3
Volume2015-January

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