Table 1.
Digestive enzymes in lysosomes
| Name | pH optimum | Related disease | Reference |
|---|---|---|---|
| beta-Galactosidase-1/GLB1 | 3.5 | GM1-gangliosidosis & Morquio B syndrome | (Zhang et al., 2000) |
| beta-Glucuronidase/GUSB | 3.5 | mucopolysaccharidosis type VII | (Shipley et al., 1993) |
| alpha-L-iduronidase/IDUA | 3.5 | mucopolysaccharidosis type I | (Scott et al., 1995) |
| Napsin A | 3.5 | – | (Aguilera et al., 2003) |
| Cathepsin D | 3.5 | – | (Barrett, 1977) |
| Cathepsin X/Z/P | 3.5 | – | (Nägler et al., 1999) |
| Chitobiase/CTBS | 3.5 | – | (Cacan et al., 1996) |
| alpha-Galactosidase A/GLA (rhGLA) | 4 | Fabry | (Ioannou et al., 1998) |
| a-N-acetylgalactosaminidase/NAGA | 4 | Schindler’s disease | (Wang et al., 1990) |
| Heparanase | 4 | – | (Vlodavsky et al., 1999) |
| Human hyaluronidase 1/HYAL1 | 4 | mucopolysaccharidosis type IX | (Hofinger et al., 2008) |
| GM1-beta-galactosidase | 4 | gangliondosis | (Norden and O’Brien, 1975) |
| β-Galactocerebrosidase | 4 | Krabbe | (Martino et al., 2009) |
| Legumain/Asparaginyl endopeptidase | 4 | – | (Chen et al., 1997) |
| Galactosylceramidase/GALC | 4.5 | Krabbe’s Disease | (Suzuki and Suzuki, 1970) |
| alpha-N-acetylglucosaminidase/NAGLU | 4.5 | Sanfilippo syndrome B | (Schmidtchen et al., 1998) |
| alpha-L-fucosidase/FUCA1 | 4.5 | fucosidosis | (Tiberio et al., 1995) |
| Hexosaminidase A/HEXA | 4.5 | Tay-Sachs disease | (Mahuran et al., 1988) |
| Human hyaluronidase 4/HYAL4 | 4.5 | – | (Kaneiwa et al., 2012) |
| alpha-Glucosidase/GAA | 4.5 | Pompe’s disease | (Wan et al., 2008) |
| sialidase | 4.5 | Sialidosis | (Jung et al., 1989) |
| Cathepsin S | 4.5 | – | (Kirschke et al., 1989) |
| Arylsulfatase A/ARSA | 4.5 | metachromatic leukodystrophy (MLD) | (Lukatela et al., 1998) |
| Sphingomyelinase | 5 | Niemann Pick | (Mühle et al., 2013) |
| Iduronate 2-sulfatase | 5 | mucopolysaccharidosis II | (Wilson et al., 1990) |
| Glucosamine (N-acetyl)-6-sulfatase | 5 | mucopolysaccharidosis type IIID | (Robertson et al., 1992) |
| N-acetylgalactosamine-6-sulfatase | 5 | mucopolysaccharidosis type IVA | (Rivera-Colón et al., 2012) |
| Hexosaminidase B/HEXB | 5.5 | Sandhoff disease | (Korneluk et al., 1986) |
| Klotho | 5.5 | – | (Tohyama et al., 2004) |
| CathepsinA | 5.5 | – | (Jackman et al., 1990) |
| Cathepsin V | 5.5 | – | (Brömme et al., 1999) |
| Arylsulfatase G/ARSG | 5.5 | mucopolysaccharidosis (in mice) | (Ferrante et al., 2002) |
| Sulfamidase | 5.5 | mucopolysaccharidosis type IIIA | (Blanch et al., 1997) |
| Glucosylceramidase/GBA | 6 | Gaucher disease | (Sorge et al., 1985) |
| Acid ceramidase | 6 | Farber | (Bedia et al., 2010) |
| Chitotriosidase/CHIT1 | 6 | – | (Aguilera et al., 2003) |
| Cathepsin B | 6 | – | (Krupa et al., 2002) |
| Cathepsin C | 6 | – | (McDonald et al., 1969) |
| Cathepsin L | 6 | – | (Mason et al., 1985) |
| Cathepsin O | 6 | – | (Mason et al., 1985) |
| Cathepsin H | 6.5 | – | (Barrett and Kirschke, 1980) |
| Cathepsin K | 6.5 | – | (Brömme et al., 1996) |
| Arylsulfatase B/ARSB | 6.5 | mucopolysaccharidosis Type VI | (Wicker et al., 1991) |
| AMSH/STAMBP | 7.2 | – | (McCullough et al., 2004) |
| beta-Glucosidase/GBA3 | 5~7 | – | (de GRAAF et al., 2001) |
| Cathepsin E | 3~7 | – | (Athauda et al., 1991) |
| Cathepsin F | 5.5~6.5 | – | (Wang et al., 1998) |
| Lysosomal acid lipase | 4 | Wolman disease | (Dairaku et al., 2014) |