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It is defined as "catalysis of the hydrolysis of terminal, non-reducing alpha-linked alpha-D-glucose residue with release of alpha-D-glucose." In this sense, "alpha-glucosidase" can encompass a wide range of enzyme activitiess, differing by the linkage of their terminal (1→3, 1→4, or 1→6), the specific identity of their substrate (sucrose ...
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] In humans, it is encoded by the GAA gene. [5]
Alpha-glucosidases are enzymes involved in breaking down complex carbohydrates such as starch and glycogen into their monomers. [2] They catalyze the cleavage of individual glucosyl residues from various glycoconjugates including alpha- or beta-linked polymers of glucose. This enzyme convert complex sugars into simpler ones.
The gene encodes a protein—acid alpha-glucosidase (EC 3.2.1.20)—which is a lysosomal hydrolase. The protein is an enzyme that normally degrades the alpha -1,4 and alpha -1,6 linkages in glycogen, maltose and isomaltose and is required for the degradation of 1–3% of cellular glycogen.
In addition to glycogen breakdown with the glycogen debranching enzyme and the glycogen phosphorylase enzyme, cells also use the enzyme acid alpha-glucosidase in lysosomes to degrade glycogen. A deficiency of an involved enzyme results in: Accumulation of glycogen in the cells; Lack of cellular energy negatively affects the involved organs
Mechanism for cleaving of alpha-1,6 linkage. Amylo-α-1,6-glucosidase (EC 3.2.1.33), or glucosidase, cleaves the remaining alpha-1,6 linkage, producing glucose and a linear chain of glycogen. [10] The mechanism by which the glucosidase cleaves the α -1,6-linkage is not fully known because the amino acids in the active site have not yet been ...
Acid alpha-glucosidase ... It may also include a non-ischemic forearm test, exercise stress test, or 12-minute walk test (12MWT). ... results in muscle ...
76051 Ensembl ENSG00000214013 ENSMUSG00000062646 UniProt Q8TET4 Q8BVW0 RefSeq (mRNA) NM_198141 NM_001301409 NM_001301410 NM_172672 RefSeq (protein) NP_001288338 NP_001288339 NP_937784 NP_766260 Location (UCSC) Chr 15: 42.27 – 42.36 Mb Chr 2: 120.23 – 120.29 Mb PubMed search Wikidata View/Edit Human View/Edit Mouse Neutral alpha-glucosidase C is an enzyme that in humans is encoded by the ...