Cell Biology 4 Lysosomal Storage Diseases

Question 1

Glycogenosis

Answer 1

Type II Pompe’s Disease
Enzyme Deficiency-alpha 1,4-Glucosidase
Metabolite Accumulation-Glycogen

Question 2

Sphingolipidosis

Answer 2

GM1 gangliosidosis
Enzyme Deficiency-GM1 ganglioside beta galactosidase
Metabolite-GM1 ganglioside, galactose-containing oligosaccharides

Question 3

Sphingolipidosis

Answer 3

GM2 gangliosidosis (Tay-Sachs)Hexosaminidase-α subunit	
GM2 ganglioside

Question 4

Sphingolipidosis

Answer 4

GM2 gangliosidosis Sandoff
Enzyme deficiency-Hexosaminidase-beta subunit
Metabolite Accumulation-GM2 ganglioside, globoside

Question 5

Sulfatidosis

Answer 5

Metachromatic leukodystrophy
Arylsulfatase A
Sulfatide

Question 6

Sufatidosis

Answer 6

Gaucher
Glucocerebrosidase
Glucocerebroside

Question 7

Mucopolysaccharidosis

Answer 7

Hurler
alpha-L-Iduronidase
Dermatan, sulfate, heparan sulfate