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α-Glucosidase hydrolyzes terminal non-reducing (1→4)-linked α-glucose residues to release a single α-glucose molecule. [ 10 ] α-Glucosidase is a carbohydrate-hydrolase that releases α-glucose as opposed to β-glucose. β-Glucose residues can be released by glucoamylase, a functionally similar enzyme.
Beta-glucosidase # EC 3.2.1.21 : is associated with Gaucher's disease: Lactase: EC 3.2.1.23 : one member of the β-galactosidase family, breaks down milk sugars, and its absence in adulthood causes lactose intolerance: Debranching enzyme # EC 3.2.1.33: in mammals, yeast and some bacteria, combines transferase and glucosidase activity in ...
Alglucosidase alfa is indicated for people with Pompe disease (GAA deficiency). [1]In 2014 the U.S. Food and Drug Administration announced the approval of alglucosidase alfa for treatment of people with infantile-onset Pompe disease, including people who are less than eight years of age.
Acid alpha-glucosidase, also called acid maltase, [5] is an enzyme that helps to break down glycogen in the lysosome. It is functionally similar to glycogen debranching enzyme , but is on a different chromosome, processed differently by the cell and is located in the lysosome rather than the cytosol. [ 6 ]
Glycoside hydrolases are classified into EC 3.2.1 as enzymes catalyzing the hydrolysis of O- or S-glycosides. Glycoside hydrolases can also be classified according to the stereochemical outcome of the hydrolysis reaction: thus they can be classified as either retaining or inverting enzymes. [6]
The human sucrase-isomaltase is a dual-function enzyme with two GH31 domains, one serving as the isomaltase, the other serving as a sucrose α-glucosidase. References [ edit ]
Glucan 1,4-α-glucosidase (EC 3.2.1.3, glucoamylase, amyloglucosidase, γ-amylase, lysosomal α-glucosidase, acid maltase, exo-1,4-α-glucosidase, glucose amylase, γ-1,4-glucan glucohydrolase, acid maltase, 1,4-α-D-glucan glucohydrolase) is an enzyme located on the brush border of the small intestine with systematic name 4-α-D-glucan glucohydrolase.
Avalglucosidase alfa is composed of the human GAA enzyme that is conjugated with a couple of bis-mannose-6-phosphate (bis-M6P) tetra-mannose glycans. [12] The bis-MGP of avalglucosidase alpha binds to the cation-independent mannose-6-phosphate receptor which is located on the skeletal muscles. [12]