Table 14-7.
Classification of Selected Lysosomal Storage Diseases That Involve the Central Nervous System of Animals
| Disease | Storage Product | Deficient Enzyme | Species | Breed |
|---|---|---|---|---|
| GM1 gangliosidosis | GM1 ganglioside | β-Galactosidase | Bovine | Holstein-Friesian |
| Canine | Beagle, English springer spaniel, Portuguese water dog, Alaskan huskies | |||
| Feline | Siamese, domestic shorthair | |||
| Ovine | Suffolk, Coopworth-Romney | |||
| GM2 gangliosidosis | GM2 ganglioside | β-Hexosaminidase | Canine | German shorthair pointer, Japanese spaniel |
| Feline | Domestic shorthair, Korat | |||
| Porcine | Yorkshire | |||
| Globoid cell leukodystrophy (Krabbe's-like disease) | Galactosylceramide (galactocerebroside) and galactosylsphingosine (psychosine) | Galactosylceramidase (galactocerebroside β-galactosidase) | Canine | West Highland terrier |
| Cairn terrier, miniature poodle, bluetick hound, beagle, Pomeranian | ||||
| Feline | Domestic shorthair, domestic longhair | |||
| Ovine | Polled Dorset | |||
| α-Mannosidosis | Mannose-containing oligosaccharide | α-Mannosidase | Bovine | Angus, Murray Grey, Galloway |
| Feline | Persian, domestic shorthair | |||
| β-Mannosidosis | Mannose-containing oligosaccharide | β-Mannosidase | Caprine | Nubian |
| Bovine | Salers | |||
| Mucopolysaccharidosis | Different glycosaminoglycans | Several different enzyme deficiencies | Canine | Plott hound (type I, Hurler's disease), miniature pinscher (type VI, Maroteaux-Lamy disease) |
| German shepherd (type VII, Sly disease) | ||||
| Feline | Domestic shorthair (type I, Hurler's disease) | |||
| Domestic shorthair, Siamese (type VI, Maroteaux-Lamy disease) | ||||
| Domestic shorthair (type VII, Sly disease) | ||||
| Caprine | Nubian (type III, Sanfilippo's disease) | |||
| Ceroid-lipofuscinosis | Subunit c of mitochondrial ATPase | Prelysosomal defect? | Canine | English setter, border collie, Tibetan terrier |
| Ovine | South Hampshire | |||
| Bovine | Devon | |||
| Sphingolipid Activating proteins A and D |
Palmitoyl protein thioesterase | Canine | Miniature schnauzer | |
| Ovine | Swedish Landrace | |||
| Unknown | Unknown | Canine | Chihuahua, cocker spaniel, Saluki, terrier-cross, blue heeler, Yugoslavian shepherd, Dalmatian, Australian cattle dog, golden retriever, dachshund, corgi | |
| Ovine | Rambouillet | |||
| Bovine | Beefmaster | |||
| Feline | Siamese, domestic shorthair | |||
| Niemann-Pick type c disease | Primarily ganglioside in neurons | Unknown | Feline Canine |
Domestic shorthair Boxer |
ATPase, Adenosine triphosphatase.