Table 1.

GSD Ia dogs described in current study.

Dog	Sex	AAV vector treatments	Age at euthanasia or death	Reason for euthanasia/death	Health abnormalities during lifetime	Previously published data
L	F	Birth: AAV2/8 (1×1013vp/kg) 36 mo: AAV2/9 (3×1012vp/kg)	8 years	Renal failure	Renal disease, elevated serum liver enzymes and cholesterol, HCA and HCC, polycystic ovarian syndrome, urolithiasis	(Koeberl et al 2008; Demaster et al 2012; Farah et al 2016)
R	M	Birth: AAV2/8 (1×1013vp/kg) 34 mo: AAV2/9 (3×1012vp/kg) 43 mo: AAV2/7 (1×1013vp/kg) 58 mo: AAV2/9 (3×1012vp/kg)	5.7 years	Renal failure	Elevated serum liver enzymes and cholesterol, severe end stage renal disease	(Koeberl et al 2008; Demaster et al 2012)
W	F	Birth: AAV2/9 (2×1013vp/kg) 15 mo: AAV2/8 (1×1012vp/kg)	7.4 years	Rapid decline in health from hemorrhage from HCC	Elevated serum liver enzymes and cholesterol, one pancreatitis event that resolved with vector therapy, HCC	(Demaster et al 2012; Brooks et al 2013; Farah et al 2016)
H	F	Birth: AAV2/9 (4×1013vp/kg) 3 mo: AAV2/7 (2×1012vp/kg) 5 mo: AAV2/8 (1×1013vp/kg)	6.9 years	Acute renal failure, pancreatitis and HCC	Elevated liver enzymes, triglycerides and cholesterol, HCA and HCC, acute renal failure and pancreatitis	(Demaster et al 2012; Brooks et al 2013; Farah et al 2016)
De	M	Birth: AAV2/9 (4×1013vp/kg) 2 mo: AAV2/7 (2×1012vp/kg) 4 mo: AAV2/8 (1×1013vp/kg)	4.1 years	Rapid decline in health likely from hemorrhage from HCC	Urolithiasis, elevated serum liver enzymes and cholesterol, HCC	(Demaster et al 2012; Brooks et al 2013)
A	F	GSD Ia carrier- not treated	Adopted	NA	Unable to breed successfully beginning at about 5 years of age, noted polycystic ovaries during ovariohysterectomy
Li	M	GSD Ia carrier-not treated	11 years	Intervertebral Disk Disease	Intervertebral Disk Disease, BUN elevated later in life