Targeting carnitine biosynthesis: Discovery of new inhibitors against γ-butyrobetaine hydroxylase

Kaspars Tars, Janis Leitans, Andris Kazaks, Diana Zelencova, Edgars Liepinsh, Janis Kuka, Marina Makrecka, Daina Lola, Viktors Andrianovs, Daina Gustina, Solveiga Grinberga, Edvards Liepinsh, Ivars Kalvinsh, Maija Dambrova, Einars Loza, Osvalds Pugovics

Research output: Contribution to journalArticlepeer-review

42 Citations (Scopus)

Abstract

γ-Butyrobetaine hydroxylase (BBOX) catalyzes the conversion of gamma butyrobetaine (GBB) to l-carnitine, which is involved in the generation of metabolic energy from long-chain fatty acids. BBOX inhibitor 3-(1,1,1-trimethylhydrazin-1-ium-2-yl)propanoate (mildronate), which is an approved, clinically used cardioprotective drug, is a relatively poor BBOX inhibitor and requires high daily doses. In this paper we describe the design, synthesis, and properties of 51 compounds, which include both GBB and mildronate analogues. We have discovered novel BBOX inhibitors with improved IC 50 values; the best examples are in the nanomolar range and about 2 orders of magnitude better when compared to mildronate. For six inhibitors, crystal structures in complex with BBOX have been solved to explain their activities and pave the way for further inhibitor design.

Original languageEnglish
Pages (from-to)2213-2236
JournalJournal of Medicinal Chemistry
Volume57
Issue number6
DOIs
Publication statusPublished - 27 Mar 2014
Externally publishedYes

Field of Science*

  • 1.6 Biological sciences
  • 3.1 Basic medicine

Publication Type*

  • 1.1. Scientific article indexed in Web of Science and/or Scopus database

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