Cutting edge: treatment of complement regulatory protein deficiency by retroviral in vivo gene therapy

J Immunol. 2006 Oct 15;177(8):4953-6. doi: 10.4049/jimmunol.177.8.4953.

Abstract

Gene therapy is an attractive means to replace a deficient or defective protein. Using a murine retroviral vector, we provide an example of reconstituting a C regulator by neonatal in vivo gene transfer. The fusion gene containing the mouse C receptor 1-related gene/protein y (Crry) and a single chain Ab fragment with specificity for mouse glycophorin A was placed under transcriptional control of a liver-specific promoter. Shortly after birth, Crry KO mice were injected with the retroviral vectors. Protein expression progressively increased over the next 6-8 wk after which an equilibrium was established. Coating levels on RBCs were obtained that inhibited C activation similar to wild-type cells and remained constant for > 1 year. Thus, gene therapy with targeted regulators represents a treatment option to provide a long-term and sustained protein supply for the site-specific blockade of undesirable complement activation.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Complement Activation / drug effects
  • Erythrocytes / immunology
  • Genetic Therapy / methods*
  • Genetic Vectors / therapeutic use
  • Glycophorins / immunology
  • Immunoglobulin Fragments / administration & dosage
  • Immunoglobulin Fragments / genetics
  • Liver
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Organ Specificity
  • Receptors, Complement / deficiency*
  • Receptors, Complement / genetics
  • Receptors, Complement 3b
  • Recombinant Fusion Proteins / administration & dosage
  • Retroviridae

Substances

  • Cr1l protein, mouse
  • Glycophorins
  • Immunoglobulin Fragments
  • Receptors, Complement
  • Receptors, Complement 3b
  • Recombinant Fusion Proteins