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. Author manuscript; available in PMC: 2019 Nov 1.
Published in final edited form as: J Inherit Metab Dis. 2018 Jul 24;41(6):965–976. doi: 10.1007/s10545-018-0223-y

Fig. 1.

Fig. 1.

Liver G6Pase persists throughout lifetime of AAV-G6Pase treated GSD Ia dogs increasing survival and ability to maintain normoglycemia during fasting. a. Hepatic G6Pase staining of AAV-G6Pase treated GSD Ia, Dog R. G6Pase is shown with brown staining. 100x b. Hepatic G6Pase staining of a GSD Ia carrier shown for comparison. 100x c. Mean ±SD of G6Pase activity detected in livers of GSD Ia dogs at end of life, AAV-G6Pase treated dogs have significantly higher G6Pase activity than untreated GSD Ia dogs , but both treated and untreated GSD Ia dogs do not achieve G6Pase activity as high as that found in carrier/wild type animals as shown through Student’s t test. Untreated, affected puppies had a low residual G6Pase activity in liver as described (Kishnani et al 2001). d. Mean ±SD of glycogen detected in GSD Ia dogs at end of life. Both treated and untreated GSD Ia dogs have increased glycogen compared with carriers and wild type through student’s t test. e. AAV-G6Pase dogs have a greatly increased survival over untreated GSD Ia dogs; as shown using a Log-rank (Mantel- Cox) test, p=0.008. f. Using the Area Under Curve (AUC), glucose curves throughout the lifetime of AAV-G6Pase treated dogs demonstrate abilities to fast and maintain normoglycemia.