Phenylquinoxalinone CFTR activator as potential prosecretory therapy for constipation

Transl Res. 2017 Apr:182:14-26.e4. doi: 10.1016/j.trsl.2016.10.003. Epub 2016 Oct 15.

Abstract

Constipation is a common condition for which current treatments can have limited efficacy. By high-throughput screening, we recently identified a phenylquinoxalinone activator of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel that stimulated intestinal fluid secretion and normalized stool output in a mouse model of opioid-induced constipation. Here, we report phenylquinoxalinone structure-activity analysis, mechanism of action, animal efficacy data in acute and chronic models of constipation, and functional data in ex vivo primary cultured human enterocytes. Structure-activity analysis was done on 175 phenylquinoxalinone analogs, including 15 synthesized compounds. The most potent compound, CFTRact-J027, activated CFTR with EC50 ∼ 200 nM, with patch-clamp analysis showing a linear CFTR current-voltage relationship with direct CFTR activation. CFTRact-J027 corrected reduced stool output and hydration in a mouse model of acute constipation produced by scopolamine and in a chronically constipated mouse strain (C3H/HeJ). Direct comparison with the approved prosecretory drugs lubiprostone and linaclotide showed substantially greater intestinal fluid secretion with CFTRact-J027, as well as greater efficacy in a constipation model. As evidence to support efficacy in human constipation, CFTRact-J027 increased transepithelial fluid transport in enteroids generated from normal human small intestine. Also, CFTRact-J027 was rapidly metabolized in vitro in human hepatic microsomes, suggesting minimal systemic exposure upon oral administration. These data establish structure-activity and mechanistic data for phenylquinoxalinone CFTR activators, and support their potential efficacy in human constipation.

MeSH terms

  • Acute Disease
  • Animals
  • Body Fluids / drug effects
  • Body Fluids / metabolism*
  • Cell Line
  • Chronic Disease
  • Constipation / drug therapy*
  • Constipation / genetics
  • Constipation / pathology
  • Cystic Fibrosis Transmembrane Conductance Regulator / metabolism*
  • Disease Models, Animal
  • Duodenum / drug effects
  • Duodenum / metabolism
  • Female
  • Gastric Acid / metabolism
  • Humans
  • Jejunum / drug effects
  • Jejunum / metabolism
  • Lubiprostone / pharmacology
  • Mice
  • Microsomes, Liver / drug effects
  • Microsomes, Liver / metabolism
  • Patch-Clamp Techniques
  • Peptides / pharmacology
  • Quinoxalines / chemical synthesis
  • Quinoxalines / chemistry
  • Quinoxalines / pharmacology
  • Quinoxalines / therapeutic use*
  • Rats
  • Scopolamine / pharmacology
  • Structure-Activity Relationship

Substances

  • Peptides
  • Quinoxalines
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • Lubiprostone
  • Scopolamine
  • linaclotide