Evaluation of optimal expression cassette in retrovirus vector for beta-thalassemia gene therapy

Mol Biotechnol. 2003 Jun;24(2):127-40. doi: 10.1385/MB:24:2:127.

Abstract

Trials of retroviral vector-mediated human beta-globin gene transfer were hampered by low titers, unstable vector transmission, and low-level expression of transferred gene. With the goal of optimizing the retrovirally encoded human beta-globin gene expression cassette for gene therapy of beta-thalassemia, we generated 3 series of vector constructs (a total of 12 constructs) and investigated the effects of the proximal promoter, 3' - enhancer, and derivatives from the beta-locus control region or alpha-major regulatory element on virus titer, vector transmission stability, and gene expression. The virus titers for 9 of the 12 vector constructs ranged between 2.8 x 10(4) cfu/mL and 1.0 x 10(6) cfu/mL. We found that proviral DNA was intact in most G418- resistant murine erythroleukemia (MEL) cell clones for 5 vector constructs, while obvious genetic instability was observed for 4 other vector constructs. MEL cells harboring the intact provirus were induced to differentiate, and human beta-globin gene expression was analyzed with RNase protection assay. The percentage of human beta-globin transcript relative to endogenous murine alpha-globin transcript were 101.8 +/- 64.3% (n = 10), 40.1 +/- 28.7% (n = 4), 31.1 +/- 31.9% (n = 12), 52.4 +/- 11.2% (n = 12), and 53.6 +/- 8.6% (n = 12) for the 5 constructs, respectively, demonstrating the development of optimized retroviral vectors for beta-globin gene therapy with murine erythroid cell lines as a model. Unexpectedly, we also documented that the point mutation 8700(C-->T) in DNase I hypersensitive site 2 (HS2) core fragment might contribute to low-level expression of the human beta-globin gene, based on a comparison of results from transfected and transduced MEL cells and sequence analysis of proviral DNA.

Publication types

  • Comparative Study
  • Evaluation Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Base Sequence
  • Cell Line, Tumor
  • Erythroid Cells / metabolism*
  • Gene Expression Regulation, Viral
  • Gene Transfer Techniques
  • Genetic Therapy / methods*
  • Genetic Vectors / genetics*
  • Globins / biosynthesis*
  • Globins / genetics*
  • Humans
  • Kidney / metabolism
  • Mice
  • Molecular Sequence Data
  • Mutagenesis, Insertional
  • NIH 3T3 Cells
  • Quality Control
  • Recombinant Proteins / genetics
  • Recombinant Proteins / metabolism
  • Retroviridae / genetics*
  • Transfection / methods
  • beta-Thalassemia / genetics*
  • beta-Thalassemia / metabolism*
  • beta-Thalassemia / therapy

Substances

  • Recombinant Proteins
  • Globins