Table 2.

Complete list of GSD animal models. * Treatment tested in cell cultures derived from the mouse model.

GSD Type	Animal Model	Naturally Occurring	Specific Model	Therapies Evaluated	References
GSD-0b	Murine model	No	Gys1-/-	Not reported	[106,107,108]
GSD-Ia/Von Gierke	Canine model	Yes	Maltase dog	Gene therapy	[109,110,111,112,113,114,115]
		Yes	Maltase-Beagle dog	Gene therapy	[111,112,113,114,115,116]
	Murine model	No	G6pc-/-	Glucose therapy Gene therapy	[112,117,118,119,120,121,122,123,124,125,126,127]
		No	L-G6pc-/-	Gene therapy RNAi approach	[128]
		No	K-G6pc-/-	Not reported	[129]
		No	I-G6pc-/-	mRNA therapy Gene therapy	[130]
GSD-Ib	Murine model	No	Slc37a4-/-	Gene therapy	[131,132,133,134]
		No	TM-Slc37a4-/-	Not reported	[135]
GSD-II Pompe	Cattle models	Yes	Shorthorn cattle	Not reported	[136,137]
		Yes	Brahman cattle	Not reported	[138,139,140,141]
	Canine model	Yes	Lapphund dog	Gene therapy	[142,143,144,145]
	Murine model	No	6neo/6neo	ERT Gene therapy	[146,147,148,149,150,151,152,153,154,155,156,157,158,159,160,161,162,163,164,165,166,167,168,169,170,171,172,173,174,175,176,177,178,179,180,181,182]
		No	AD-6neo/6neo	Not reported	[183,184]
		No	AD2-6neo/6neo	Not reported	[185]
		No	Δ6/Δ6	Not reported	[146,150]
		No	13neo/13neo	Not reported	[186]
		No	Δ14neo/Δ14neo	Not reported	[150]
		No	GaaKODBA	Not reported	[151]
		No	Gaac1826dupA	Not reported	[183]
	Quail models	Yes	Japanese quails	ERT	[187,188,189,190]
GSD-III/Cori	Canine model	Yes	Curly-coated retrievers (CCR)	Not reported	[191,192,193]
	Murine model	No	AglEX5-/-	RNAi approach	[49]
		No	AglEX32-/-		[194]
		No	AglEX6-10-/-	Gene therapy	[195]
		No	AglEX6-10-/-2	Gene therapy	[196]
GSD-IV/Andersen	Horse model	Yes	American Quarter Horse foals (AQHs)	Not reported	[197]
	Cat Model	Yes	Norwegian forest cats	Not reported	[198]
	Murine Model	No	Gbe1neo/neo	Not reported	[199]
		No	Gbe1-/-	Not reported	[199]
		No	Gbe1ys/ys	Gene therapy	[200,201]
GSD-V/McArdle	Bovine model	Yes	Charolais cattle	Not reported	[202,203]
	Ovine model	Yes	Merino sheep	VPA treatment Gene therapy	[204,205,206,207]
	Murine model	No	Pygmp.R50X/p.R50X	VPA treatment Read-through * Gene therapy	[208,209,210,211,212,213,214]
	Zebrafish model	No	N.A.	Not reported	[215]
GSD-VI/Hers	Murine model	No	Pygl-/-	Not reported	[216]
GSD-VII/Tauri	Canine models	Yes	English Springer Spaniel	Not reported	[217,218,219,220,221,222,223,224]
		Yes	American Cocker Spaniels	Not reported	[217,218,219,220,221,222,223,224]
		Yes	Whippet	Not reported	[217,218,219,220,221,222,223,224]
		Yes	Wachtelhunds	Not reported	[225,226]
	Murine model	No	Pfkm-/-	Not reported	[227]
GSD-XV	Murine model	No	Gyg-/-	Not reported	[228]