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ARSA | arylsulfatase A; Hydrolyzes cerebroside sulfate (509 aa) | |||
GLA | galactosidase, alpha (429 aa) | |||
GALK1 | galactokinase 1; Major enzyme for galactose metabolism (392 aa) | |||
NEU2 | sialidase 2 (cytosolic sialidase); Hydrolyzes sialylated compounds (380 aa) | |||
GALC | galactosylceramidase; Hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon (685 aa) | |||
HEXB | hexosaminidase B (beta polypeptide); Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues (556 aa) | |||
LCT | lactase; LPH splits lactose in the small intestine (1927 aa) | |||
SI | sucrase-isomaltase (alpha-glucosidase); Plays an important role in the final stage of carbohydrate digestion. Isomaltase activity is specific for both alpha-1,4- and alpha-1,6-oligosaccharides (1827 aa) | |||
HEXA | hexosaminidase A (alpha polypeptide); Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. The form B is active against certain oligosaccharides. The form S has no measurable activity (529 aa) | |||
GALM | galactose mutarotase (aldose 1-epimerase); Mutarotase converts alpha-aldose to the beta-anomer. It is active on D-glucose, L-arabinose, D-xylose, D-galactose, maltose and lactose (By similarity) (342 aa) | |||
AKR1B1 | aldo-keto reductase family 1, member B1 (aldose reductase); Catalyzes the NADPH-dependent reduction of a wide variety of carbonyl-containing compounds to their corresponding alcohols with a broad range of catalytic efficiencies (316 aa) | |||
NEU3 | sialidase 3 (membrane sialidase); Plays a role in modulating the ganglioside content of the lipid bilayer at the level of membrane-bound sialyl glycoconjugates (461 aa) | |||
GAA | glucosidase, alpha; acid; Essential for the degradation of glygogen to glucose in lysosomes (952 aa) | |||
B4GALT6 | UDP-Gal-betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 6; Required for the biosynthesis of glycosphingolipids (382 aa) | |||
GLB1 | galactosidase, beta 1; Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans (677 aa) | |||
UGT8 | UDP glycosyltransferase 8; Catalyzes the transfer of galactose to ceramide, a key enzymatic step in the biosynthesis of galactocerebrosides, which are abundant sphingolipids of the myelin membrane of the central nervous system and peripheral nervous system (541 aa) | |||
NEU4 | sialidase 4; May function in lysosomal catabolism of sialylated glycoconjugates. Has sialidase activity towards synthetic substrates, such as 2’-(4-methylumbelliferyl)-alpha-D-N- acetylneuraminic acid (4-MU-NANA or 4MU-NeuAc). Has a broad substrate specificity being active on glycoproteins, oligosaccharides and sialylated glycolipids (497 aa) | |||
B3GALNT1 | beta-1,3-N-acetylgalactosaminyltransferase 1 (globoside blood group); Transfers N-acetylgalactosamine onto globotriaosylceramide (331 aa) | |||
GANC | glucosidase, alpha; neutral C; Has alpha-glucosidase activity (914 aa) | |||
GAL3ST1 | galactose-3-O-sulfotransferase 1; Catalyzes the sulfation of membrane glycolipids. Seems to prefer beta-glycosides at the non-reducing termini of sugar chains attached to a lipid moiety. Catalyzes the synthesis of galactosylceramide sulfate (sulfatide), a major lipid component of the myelin sheath and of monogalactosylalkylacylglycerol sulfate (seminolipid), present in spermatocytes (By similarity). Also acts on lactosylceramide, galactosyl 1-alkyl-2-sn-glycerol and galactosyl diacylglycerol (in vitro) (423 aa) | |||
AKR1B10 | aldo-keto reductase family 1, member B10 (aldose reductase); Acts as all-trans-retinaldehyde reductase. Can efficiently reduce aliphatic and aromatic aldehydes, and is less active on hexoses (in vitro). May be responsible for detoxification of reactive aldehydes in the digested food before the nutrients are passed on to other organs (316 aa) | |||
GBP2 | guanylate binding protein 2, interferon-inducible; Hydrolyzes GTP to GMP in two consecutive cleavage reactions. Exhibits antiviral activity against influenza virus. Promote oxidative killing and deliver antimicrobial peptides to autophagolysosomes, providing broad host protection against different pathogen classes (By similarity) (591 aa) | |||
NEU1 | sialidase 1 (lysosomal sialidase) (415 aa) | |||
OTUD4 | OTU domain containing 4 (1049 aa) | |||
MGAM | maltase-glucoamylase (alpha-glucosidase); May serve as an alternate pathway for starch digestion when luminal alpha-amylase activity is reduced because of immaturity or malnutrition. May play a unique role in the digestion of malted dietary oligosaccharides used in food manufacturing (1857 aa) | |||
GALK2 | galactokinase 2; Acts on GalNAc. Also acts as a galactokinase when galactose is present at high concentrations. May be involved in a salvage pathway for the reutilization of free GalNAc derived from the degradation of complex carbohydrates (458 aa) |