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

Contents:

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
BRENDA 3.2.1.20
ChEMBL Target CHEMBL2608 (Hs), CHEMBL1667668 (Mm), CHEMBL3513 (Rn)
DrugBank Target P10253 (Hs)
Ensembl Gene ENSG00000171298 (Hs), ENSMUSG00000025579 (Mm), ENSRNOG00000047656 (Rn)
Entrez Gene 2548 (Hs), 14387 (Mm), 367562 (Rn)
Human Protein Atlas ENSG00000171298 (Hs)
KEGG Enzyme 3.2.1.20
KEGG Gene hsa:2548 (Hs), mmu:14387 (Mm), rno:367562 (Rn)
OMIM 606800 (Hs)
Orphanet ORPHA121987 (Hs)
RefSeq Nucleotide NM_000152 (Hs), NM_008064 (Mm), NM_199118 (Rn)
RefSeq Protein NP_000143 (Hs), NP_032090 (Mm), NP_954549 (Rn)
UniProtKB P10253 (Hs), P70699 (Mm), Q6P7A9 (Rn)
Wikipedia GAA (Hs)
Enzyme Reaction
EC Number: 3.2.1.20

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

Inhibitors
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].
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.

References

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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. Bioorganic & Medicinal Chemistry Letters, 7 (3): 355-360.

How to cite this page

3.2.1.- Glycosidases: glucosidase alpha, acid. Last modified on 23/01/2019. Accessed on 12/12/2019. IUPHAR/BPS Guide to PHARMACOLOGY, http://www.guidetopharmacology.org/GRAC/ObjectDisplayForward?objectId=2611.