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. Author manuscript; available in PMC: 2020 May 1.
Published in final edited form as: J Inherit Metab Dis. 2019 Feb 22;42(3):470–479. doi: 10.1002/jimd.12069

Fig. 3.

Fig. 3.

Transient expression assays of G6Pase-α constructs and phenotype analyses of rAAV-treated G6pc−/− mice. (a) Phosphohydrolase activity after transfecting the pSVL-hG6Pase-α-WT, pSVL-hG6Pase-α-S298C, pSVL-co-hG6Pase-α, or pSVL-co-hG6Pase-α-S298C construct into COS-1 cells. (b) Western-blot analysis of hG6Pase-α and β-actin after transient expression of the pSVL-hG6Pase-α-WT, pSVL-hG6Pase-α-S298C, pSVL-co-hG6Pase-α, pSVL-co-hG6Pase-α-S298C construct into COS-1 cell along with quantification by densitometry (n = 3). (c-f) Phenotypic analyses of rAAV-treated G6pc−/− mice. Two-week-old G6pc−/− mice were treated with 1012 or 1013 vp/kg of rAAV-WT (n = 12), rAAV-S298C (n = 12), rAAV-co (n = 12), or rAAV-co-S298C (n = 12) vector, and phenotype analyzed at age 4 weeks (6 mice/treatment) and 12 weeks (6 mice/treatment). (c) Hepatic microsomal G6Pase-α activity in mice treated with the rAAV vectors at 1012 vp/kg. (d) Vector genome copy numbers in the livers of 12-week-old mice. (e) Hepatic microsomal G6Pase-α activity in mice treated with the rAAV vectors at 1013 vp/kg. (f) Fasting blood glucose tolerance profiles and blood glucose levels following a 24-hour of fast. Data represent the mean ± SEM. *p < 0.05, **p < 0.005.