Table 1.

Companion Animal Models of Lysosomal Storage Diseases.

Lysosomal Storage Disease Subgroup	Disease	Defective Gene	Mutation^Ref	Affected Species; Breeds
Glycoproteinoses
	Alpha-mannosidosis	MAN2B1	c.1748delCCAG (Berg et al., 1997)	Feline; DLH, DSH, Persian
	Fucosidosis	FUCA1	c.379_392del14bp (Skelly et al., 1996)	Canine; English Springer Spaniel
Mucopolysaccharidoses
	Mucopolysaccharidosis I	IDUA	c.155 + 1G > A (Menon et al., 1992)c.1107– 1109 del (He et al., 1999)	Canine; Plott Hound Feline; DSH
	Mucopolysaccharidosis IIIA	SGSH	c.708–709insA (Yogalingam et al., 2002)	Canine; New Zealand Huntaway dogs
	Mucopolysaccharidosis IIIB	NAGLU	Unpublished	Canine; Schipperke
	Mucopolysaccharidosis VI	ARSB	c.1427T > C (Yogalingam et al., 1996)	Feline; Siamese
	Mucopolysaccharidosis VII	GUSB	c.559 G > A (Ray et al., 1998)c.1074G > A (Fyfe et al., 1999)	Canine; Mixed breed Feline; DSH
Oligosaccharidoses
	Pompe’s disease	GAA	c.2237 G > A (Seppala et al., 2013)	Canine; Swedish and Finnish Lapphunds
Proteinoses
	Ceroid Lipofuscinosis	CLN5	c.934_935delAG (Gilliam et al., 2015)	Canine; Golden Retriever
Sphingolipidoses
	Globoid cell leukodystrophy	GALC	c.473A > C (Victoria et al., 1996)	Canine; West Highland White and Cairn terriers
	GM₁-gangliosidosis	GLB1	c.1647delC (Yamato et al., 2002)	Canine; Shiba Inu
	GM₂-gangliosidosis	HEXA	c.967 G > A (Sanders et al., 2013)	Canine; Japanese Chin
	Niemann Pick Type C1	NPC1	c.2864 G > C (Somers et al., 2003)	Feline; DSH

DLH = Domestic long-hair cat.

DSH = Domestic short-hair cat.