Long-term safety and efficacy of AAV gene therapy in the canine model of glycogen storage disease type Ia.
Young Mok LeeThomas J ConlonAndrew SpechtKirsten E ColemanLaurie M BrownAna M EstrellaMonika DambskaKathryn R DahlbergWeinstein DAPublished in: Journal of inherited metabolic disease (2018)
rAAV-GPE-hG6PC treatment in GSD-Ia dogs was found to be safe and efficacious. GSD-Ia is an attractive target for human gene therapy since it is a monogenic disorder with limited tissue involvement. Blood glucose and lactate monitoring can be used to assess effectiveness and as a biomarker of success. GSD-Ia can also serve as a model for other hepatic monogenic disorders.