D-Penicillamine modulates hydrogen sulfide (H2S) pathway through selective inhibition of cystathionine-γ-lyase

Br J Pharmacol. 2016 May;173(9):1556-65. doi: 10.1111/bph.13459. Epub 2016 Mar 23.

Abstract

Background and purpose: Hydrogen sulfide (H2S) is a gasotransmitter produced from L-cysteine through the enzymatic action of cystathionine-γ-lyase (CSE) and/or cystathionine-β-synthase. D-Penicillamine is the d isomer of a dimethylated cysteine and has been used for the treatment of rheumatoid arthritis. AsD-penicillamine is structurally very similar to cysteine, we have investigated whether D-penicillamine, as a cysteine analogue, has an effect on the H2 S pathway.

Experimental approach: We tested the effect of D-penicillamine (0.01-1 mM) in mouse aortic rings mounted in isolated organ baths and determined whether it could affect H2 S biosynthesis. In particular, we investigated any possible inhibitor or donor behaviour by using recombinant enzyme-based assays and an in vivo approach.

Key results: D-Penicillamine, per se, showed little or no vasodilator effect, and it cannot be metabolized as a substrate in place of l-cysteine. However, d-penicillamine significantly reduced L-cysteine-induced vasodilatation in a concentration-dependent manner through inhibition of H2 S biosynthesis, and this effect occurred at concentrations 10 times lower than those needed to induce the release of H2 S. In particular, D-penicillamine selectively inhibited CSE in a pyridoxal-5'-phosphate-dependent manner.

Conclusions and implications: Taken together, our results suggest that D-penicillamine acts as a selective CSE inhibitor, leading to new perspectives in the design and use of specific pharmacological tools for H2 S research. In addition, the inhibitory effect of D-penicillamine on CSE could account for its beneficial action in rheumatoid arthritis patients, where H2 S has been shown to have a detrimental effect.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cystathionine gamma-Lyase / antagonists & inhibitors*
  • Cystathionine gamma-Lyase / metabolism
  • Dose-Response Relationship, Drug
  • Enzyme Inhibitors / chemistry
  • Enzyme Inhibitors / pharmacology*
  • Hydrogen Sulfide / metabolism*
  • Male
  • Mice
  • Penicillamine / chemistry
  • Penicillamine / pharmacology*
  • Signal Transduction / drug effects*
  • Structure-Activity Relationship

Substances

  • Enzyme Inhibitors
  • Cystathionine gamma-Lyase
  • Penicillamine
  • Hydrogen Sulfide