Table 1. Lysosomal storage disorders.

Disease	Defective protein	Major storage materials
Sphingolipidoses
Gaucher disease	GBA/acid β-glucosidase	Glucosylceramide
Fabry disease	GLA/α-galactosidase A	Globotriaosylceramide
GM1 gangliosidosis	GLB1/β-galactosidase	GM1 ganglioside
Niemann-Pick type C disease	NPC1 or NPC2	Cholesterol and sphingolipids
Mucopolysaccharidoses (MPS)
MPS IIIA (Sanfilippo syndrome)	SGSH/N-sulfoglucosamine sulfohydrolase	Heparan sulfate
MPS VI (Maroteaux–Lamy syndrome)	ARSB/arylsulfatase B	Dermatan sulfate
Multiple sulfatase deficiency	SUMF1/sulfatase modifying factor 1	Sulfatide and glycosaminoglycans
Mucolipidoses (ML)
ML II (I-cell disease)	GNPTAB/N-acetylglucosamine-1-phosphate transferase	Glycosaminoglycans and lipids
ML III	GNPTAB/N-acetylglucosamine-1-phosphate transferase	Glycosaminoglycans and lipids
ML IV	MCOLN1/mucolipin 1	Glycosaminoglycans and lipids
Glycogen storage disease
Pompe disease (glycogen storage disease type II)	GAA/acid α-glucosidase	Glycogen
Danon disease	LAMP2	Glycogen and cytoplasmic debris
Neuronal ceroid lipofuscinoses (CLN)
Batten disease (JNCL)	CLN3/battenin	Subunit c of mitochondrial ATP synthase
CLN 10	CTSD/cathepsin D	Granular osmiophilic deposits

This table shows examples of lysosomal storage diseases that are accompanied by accumulation of autophagic vesicles.