Correction of purine nucleoside phosphorylase deficiency by retroviral-mediated gene transfer in mouse S49 T cell lymphoma: a model for gene therapy of T cell immunodeficiency

Hum Gene Ther. 1992 Dec;3(6):625-31. doi: 10.1089/hum.1992.3.6-625.

Abstract

To determine the effectiveness of retroviral-mediated purine nucleoside phosphorylase (PNP) gene transfer and expression for metabolic correction of PNP deficiency, we used as a gene transfer target the NSU-1 subline of murine S49 T lymphoma cells, an in vitro genetic model of PNP deficiency. NSU-1 cells were transduced with recombinant retroviruses that express either the murine or human PNP coding sequences under transcriptional regulation of the Moloney murine leukemia virus (Mo-MLV) long terminal repeat (LTR), resulting in expression of substantial levels of PNP activity. Untransduced or control virus-transduced NSU-1 cells were extremely sensitive to deoxyguanosine, a PNP substrate that is toxic for lymphoid cells. However, PNP-virus transduction of NSU-1 cells metabolically corrected the sensitivity of these cells to deoxyguanosine, resulting in near wild-type levels of growth inhibition. These results demonstrate that retroviral-mediated PNP gene transfer and expression corrects the metabolic defect observed in PNP-deficient murine lymphoid cells, suggesting that PNP gene transfer and expression in human lymphoid cells might similarly correct substrate-mediated toxicity and provide an effective genetic therapy.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Deoxyguanosine / pharmacology
  • Drug Resistance
  • Enzyme Induction
  • Genetic Therapy*
  • Genetic Vectors
  • Immunologic Deficiency Syndromes / genetics
  • Immunologic Deficiency Syndromes / therapy*
  • Lymphoma, T-Cell / pathology*
  • Mice
  • Moloney murine leukemia virus / genetics
  • Promoter Regions, Genetic
  • Purine-Nucleoside Phosphorylase / deficiency*
  • Recombinant Fusion Proteins / metabolism
  • T-Lymphocytes / drug effects
  • T-Lymphocytes / enzymology*
  • Transduction, Genetic
  • Tumor Cells, Cultured / drug effects
  • Tumor Cells, Cultured / enzymology

Substances

  • Recombinant Fusion Proteins
  • Purine-Nucleoside Phosphorylase
  • Deoxyguanosine