FIG. 1.

Prevention of hypoglycemia following neonatal AAV2/9-G6Pase administration. Blood glucose was determined every 2 hr following fasting of duration indicated. (A) Dog W was treated with AAV2/9-G6Pase at 2 days of age (2×10¹³ vp/kg), and subsequently fasted for 8 hr at the indicated ages. (B) Puppies Du, F, and Ro were treated with AAV2/9-G6Pase at 2 days of age (4×10¹³ vp/kg), and subsequently fasted for 8 hr at 2–3 weeks of age. These three puppies did not survive past 3 months of age. (C) Puppies De, H, and T were treated with AAV2/9-G6Pase at 2 days of age (4×10¹³ vp/kg), and subsequently fasted for 8 hr at 2–3 weeks of age. These three puppies were re-treated with AAV2/7- and AAV2/8-G6Pase as a group to prevent mortality (Table 1). Glucose curves for normal puppies (n=10) and GSD-Ia puppies treated with dietary therapy alone (n=4) are shown for comparison. AAV, adeno-associated virus; AAV2, adeno-associated virus serotype 2; G6Pase, glucose-6-phosphatase; GSD-Ia, glycogen storage disease type Ia; vp, vector particles. Color images available online at www.liebertonline.com/hum