glucosidase alpha, acid | 3.2.1.- Glycosidases | IUPHAR/BPS Guide to PHARMACOLOGY

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glucosidase alpha, acid

Target not currently curated in GtoImmuPdb

Target id: 2611

Nomenclature: glucosidase alpha, acid

Family: 3.2.1.- Glycosidases

Annotation status:  image of a grey circle Awaiting annotation/under development. Please contact us if you can help with annotation.  » Email us

Gene and Protein Information
Species TM AA Chromosomal Location Gene Symbol Gene Name Reference
Human - 952 17q25.2-q25.3 GAA glucosidase alpha, acid
Mouse - 953 11 D-E Gaa glucosidase, alpha, acid
Rat - 953 10 Gaa glucosidase, alpha, acid
Previous and Unofficial Names
lysosomal alpha-glucosidase | glucosidase, alpha, acid | glucosidase, alpha; acid | glucosidase
Database Links
ChEMBL Target
DrugBank Target
Ensembl Gene
Entrez Gene
Human Protein Atlas
KEGG Enzyme
RefSeq Nucleotide
RefSeq Protein
Enzyme Reaction
EC Number:

Download all structure-activity data for this target as a CSV file

Key to terms and symbols View all chemical structures Click column headers to sort
Ligand Sp. Action Value Parameter Reference
1-deoxynojirimycin Hs Inhibition 6.8 pKi 4
pKi 6.8 (Ki 1.5x10-7 M) [4]
migalastat Hs Binding 7.4 pIC50 1
pIC50 7.4 (IC50 4x10-8 M) [1]
miglitol Hs Inhibition 6.5 pIC50 3
pIC50 6.5 (IC50 3.5x10-7 M) [3]
Inhibitor Comments
1-deoxynojirimycin also inhibits the related enzyme, α-glucosidase neutral AB (GANAB; Q14697) with an IC50 value of 1300nM [2].
Immuno Process Associations
Immuno Process:  Inflammation
GO Annotations:  Associated to 1 GO processes
GO:0043312 neutrophil degranulation TAS
Immuno Process:  Cellular signalling
GO Annotations:  Associated to 1 GO processes
GO:0043312 neutrophil degranulation TAS
Clinically-Relevant Mutations and Pathophysiology
Disease:  Glycogen storage disease II; GSD2
Synonyms: Glycogen storage disease due to acid maltase deficiency [Orphanet: ORPHA365]
Glycogen storage disease due to acid maltase deficiency, adult onset [Orphanet: ORPHA308604]
Glycogen storage disease due to acid maltase deficiency, infantile onset [Orphanet: ORPHA308552]
Glycogen storage disease due to acid maltase deficiency, juvenile onset [Orphanet: ORPHA308573]
Pompe disease
Disease Ontology: DOID:2752
OMIM: 232300
Orphanet: ORPHA308604, ORPHA365, ORPHA308552, ORPHA308573
General Comments
GAA is one member of the α-glucosidase family of enzymes which break down starch and disaccharides to glucose. α-glucosidase inhibitors are used as adjuncts to help regulate glucose levels in patients with type 2 diabetes mellitus.


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1. Asano N, Ishii S, Kizu H, Ikeda K, Yasuda K, Kato A, Martin OR, Fan JQ. (2000) In vitro inhibition and intracellular enhancement of lysosomal alpha-galactosidase A activity in Fabry lymphoblasts by 1-deoxygalactonojirimycin and its derivatives. Eur. J. Biochem., 267 (13): 4179-86. [PMID:10866822]

2. Asano N, Oseki K, Kizu H, Matsui K. (1994) Nitrogen-in-the-ring pyranoses and furanoses: structural basis of inhibition of mammalian glycosidases. J. Med. Chem., 37 (22): 3701-6. [PMID:7966130]

3. Kuriyama C, Kamiyama O, Ikeda K, Sanae F, Kato A, Adachi I, Imahori T, Takahata H, Okamoto T, Asano N. (2008) In vitro inhibition of glycogen-degrading enzymes and glycosidases by six-membered sugar mimics and their evaluation in cell cultures. Bioorg. Med. Chem., 16 (15): 7330-6. [PMID:18595718]

4. Lesur B, Ducep J-B, Lalloz M-N, Ehrhard A, Danzin C. (1997) New deoxynojirimycin derivatives as potent inhibitors of intestinal α-glucohydrolases. Bioorg. Med. Chem. Lett., 7 (3): 355-360.

How to cite this page

3.2.1.- Glycosidases: glucosidase alpha, acid. Last modified on 23/01/2019. Accessed on 07/07/2020. IUPHAR/BPS Guide to PHARMACOLOGY,