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Gene and Protein Information ![]() |
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Species | TM | AA | Chromosomal Location | Gene Symbol | Gene Name | Reference |
Human | - | 952 | 17q25.3 | GAA | glucosidase alpha, acid | |
Mouse | - | 953 | 11 83.35 cM | Gaa | glucosidase, alpha, acid | |
Rat | - | 953 | 10q32.3 | Gaa | glucosidase, alpha, acid |
Previous and Unofficial Names ![]() |
lysosomal alpha-glucosidase | glucosidase, alpha, acid | glucosidase, alpha; acid | glucosidase |
Database Links ![]() |
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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) |
Pharos | P10253 (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 ![]() |
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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 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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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 | |||||||||
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Clinically-Relevant Mutations and Pathophysiology ![]() |
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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. |
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.
3.2.1.- Glycosidases: glucosidase alpha, acid. Last modified on 23/01/2019. Accessed on 28/02/2021. IUPHAR/BPS Guide to PHARMACOLOGY, http://www.guidetopharmacology.org/GRAC/ObjectDisplayForward?objectId=2611.