What is the substrate of alpha-glucosidase?

What is the substrate of alpha-glucosidase?

What is the substrate of alpha-glucosidase?

The neutral α-glucosidase from porcine serum appeared very substrate-specific. It hydrolyzes maltooligosaccharides, phenyl α-maltoside, nigerose, soluble starch, amylose, amylopectin, and β-limit dextrins.

What does acid alpha glucosidase do?

Acid alpha-glucosidase is the enzyme responsible for degradation of glycogen polymers to glucose in the acidic milieu of the lysosomes. Cardiac and skeletal muscles are the two major tissues affected by the accumulation of glycogen within the lysosomes.

Where is acid alpha glucosidase produced?

Acid α-glucosidase (GAA) Deficiency (GSDII), or Pompe Disease, is the only glycogen storage disease directly involving abnormal lysosomal metabolism. GAA (also known as acid maltase) is an α-amylase that is localized exclusively to the lysosome.

Where is GAA produced?

the lysosome
GAA is an enzyme that catalyzes the breakdown of glycogen into glucoses in the acidic milieu of the lysosome. In human cells, GAA is produced as 110 kDa precursor, 95 kDa intermediate, and 76/70 kDa mature glycoproteins (Moreland et al., 2005).

What kind of macromolecule is alpha-glucosidase?

This is in contrast to beta-glucosidase. Alpha-glucosidase breaks down starch and disaccharides to glucose. Maltase, a similar enzyme that cleaves maltose, is nearly functionally equivalent….

Alpha-glucosidase
PDB structures RCSB PDB PDBe PDBsum
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What do alpha glucosidase inhibitors do?

Alpha-glucosidase inhibitors lower blood glucose by modifying the intestinal absorption of carbohydrates.

What kind of macromolecule is the enzyme alpha-glucosidase?

Alpha-glucosidase hydrolyzes terminal non-reducing (1→4)-linked alpha-glucose residues to release a single alpha-glucose molecule. Alpha-glucosidase is a carbohydrate-hydrolase that releases alpha-glucose as opposed to beta-glucose. Beta-glucose residues can be released by glucoamylase, a functionally similar enzyme.

What is GAA biology?

Normal Function. Collapse Section. The GAA gene provides instructions for producing an enzyme called acid alpha-glucosidase (also known as acid maltase). This enzyme is active in lysosomes, which are structures that serve as recycling centers within cells.

What is the primary structure of alpha-glucosidase?

According to the constructed model, human acid α-glucosidase is composed of five domains; that is, a trefoil type-P domain (residues 89–135), an N-terminal β-sandwich domain (residues 136–346), a catalytic (β/α)8 barrel domain (residues 347–723) with two inserted loops, which include insert 1 (residues 444–491) and …

What is the mechanism of action of alpha-glucosidase inhibitors quizlet?

Glucose, because Alpha-Glucosidase Inhibitors inhibit the enzyme that turns disaccharides to monosaccharides, so if you gave them sucrose, they can’t convert it into a monosaccharide so they can’t absorb it.

What is acid alpha glucosidase?

Acid alpha-glucosidase. 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. In humans, it is encoded by the GAA gene. Errors in this gene cause glycogen storage disease type II (Pompe disease).

What is the substrate of α-glucosidase?

Corn α-glucosidase splits glucose from starch as the sole product, with no intermediary compounds. 1159,1160 Two glucosidases were isolated from rice. 1157,1161 They were strongly activated by KCl and by monovalent and divalent cations. With maltose as the substrate, such activation did not occur and glucose did not inhibit the enzyme activity.

Is acid α-glucosidase a competitive enzyme?

Acid α-glucosidase was purified 3500-fold from rabbit muscle. 2. The enzyme was activated by cations, the degree of activation varying with the substrate. Enzyme action on glycogen was most strongly activated and activation was apparently of a non-competitive type.

What is the function of the alpha-glucosidase gene?

This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing.