Purine nucleoside phosphorylase inhibitor BCX-1777 (Immucillin-H)--a novel potent and orally active immunosuppressive agent

Int Immunopharmacol. 2001 Jun;1(6):1199-210. doi: 10.1016/s1567-5769(01)00056-x.

Abstract

Patients with purine nucleoside phosphorylase (PNP) deficiency present a selective T-cell immunodeficiency. Inhibitors of PNP are, therefore, of interest as potential T-cell selective immunosuppressive agents. BCX-1777 is a potent inhibitor of PNP from various species including human, mouse, rat, monkey and dog, with IC50 values ranging from 0.48 to 1.57 nM. BCX-1777, in the presence of 2'-deoxyguanosine (dGuo, 3-10 microM), inhibits human lymphocyte proliferation activated by various agents such as interleukin-2 (IL-2), mixed lymphocyte reaction (MLR) and phytohemagglutinin (PHA) (IC50 values < 0.1-0.38 microM). BCX-1777 is a 10-100-fold more potent inhibitor of human lymphocyte proliferation than other known PNP inhibitors like PD141955 and BCX-34. Nucleotide analysis of human lymphocytes indicate that inhibition of proliferation by BCX-1777 correlates with dGTP levels in the cells. BCX-1777 has excellent oral bioavailability (63%) in mice. At a single dose of 10 mg/kg in mice, BCX-1777 elevates dGuo to approximately 5 microM. BCX-1777 was not effective in mouse T-cell models such as delayed type hypersensitivity (DTH) and splenomegaly because mouse T-cells do not accumulate dGTP as do human T-cells. However, in the human peripheral blood lymphocyte severe combined immunodeficiency (hu-PBL-SCID) mouse model, BCX-1777 was effective in prolonging the life span 2-fold or more. This is the first known example of a PNP inhibitor that elevates dGuo in mice similar to the levels observed in PNP-deficient patients. Furthermore, these dGuo levels are also required for in vitro T-cell inhibition by BCX-1777. Thus, BCX-1777 represents a novel class of selective immunosuppressive agents that could have therapeutic utility in various T-cell disorders.

MeSH terms

  • Administration, Oral
  • Animals
  • Biological Availability
  • Enzyme Inhibitors / pharmacokinetics
  • Enzyme Inhibitors / pharmacology*
  • Graft vs Host Reaction / drug effects
  • Guanosine Triphosphate / metabolism
  • Immunosuppressive Agents / pharmacology*
  • Indicators and Reagents
  • Injections, Intravenous
  • Lymphocyte Culture Test, Mixed
  • Mice
  • Mice, Inbred BALB C
  • Mice, SCID
  • Purine Nucleosides
  • Purine-Nucleoside Phosphorylase / antagonists & inhibitors*
  • Pyrimidinones / pharmacokinetics
  • Pyrimidinones / pharmacology*
  • Pyrroles / pharmacokinetics
  • Pyrroles / pharmacology*
  • Survival Analysis
  • T-Lymphocytes / drug effects
  • T-Lymphocytes / immunology

Substances

  • Enzyme Inhibitors
  • Immunosuppressive Agents
  • Indicators and Reagents
  • Purine Nucleosides
  • Pyrimidinones
  • Pyrroles
  • forodesine
  • Guanosine Triphosphate
  • Purine-Nucleoside Phosphorylase