A universal strategy for AAV delivery of base editors to correct genetic point mutations in neonatal PKU mice

Feb 10, 2022Molecular therapy. Methods & clinical development

A universal method using viral delivery to fix genetic mutations in newborn mice with PKU

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Abstract

Up to 27.7% correction of a phenylketonuria mutation was achieved using a dual adeno-associated virus strategy.

  • Base editing tools can convert C:G or A:T base pairs to T:A or G:C, targeting specific genetic lesions.
  • The efficiency of correcting disease-causing mutations has been limited by the size of existing base editors.
  • A novel dual AAV strategy was designed to deliver base editors, linking deaminases to Cas9 using a specific peptide-antibody interaction.
  • One or two copies of the GCN4 peptide were sufficient for assembling base editors, resulting in effective targeted editing.
  • The dual AAV system successfully corrected PKU-related mutations in neonatal mice, alleviating symptoms of hyperphenylalaninemia.

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