Literature summary for 3.2.1.20 extracted from

Yoshimizu, M.; Tajima, Y.; Matsuzawa, F.; Aikawa, S.; Iwamoto, K.; Kobayashi, T.; Edmunds, T.; Fujishima, K.; Tsuji, D.; Itoh, K.; Ikekita, M.; Kawashima, I.; Sugawara, K.; Ohyanagi, N.; Suzuki, T.; Togawa, T.; Ohno, K.; Sakuraba, H.
Binding parameters and thermodynamics of the interaction of imino sugars with a recombinant human acid alpha-glucosidase (alglucosidase alfa): insight into the complex formation mechanism (2008), Clin. Chim. Acta, 391, 68-73.

Application

Application	Comment	Organism
medicine	combination of biochemical and structural investigations will give a lot of information for developing enzyme enhancement therapy (EET) for Pompe disease	Homo sapiens

Inhibitors	Comment	Organism
1-deoxynojirimycin	competitive inhibition, binds strongly to the enzyme	Homo sapiens
additional information	inhibitory and binding effects of four imino sugars on a recombinant human acid alpha-glucosidase, alglucosidase alfa, by means of inhibition assaying and isothermal titration calorimetry (ITC)	Homo sapiens
N-butyl-deoxynojirimycin	competitive inhibition	Homo sapiens
N-ethyl-deoxynojirimycin	competitive inhibition	Homo sapiens
N-methyl-deoxynojirimycin	competitive inhibition	Homo sapiens

KM Value [mM]	KM Value Maximum [mM]	Substrate	Comment	Organism	Structure
1.4	-	4-methylumbelliferyl alpha-D-glucopyranoside	-	Homo sapiens

Organism	UniProt	Comment	Textmining
Homo sapiens	P10253	-	-

Specific Activity Minimum [µmol/min/mg]	Specific Activity Maximum [µmol/min/mg]	Comment	Organism
4.72	-	4-methylumbelliferyl alpha-D-glucopyranoside as a substrate	Homo sapiens

Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
4-methylumbelliferyl alpha-D-glucopyranoside + H2O	-	Homo sapiens	4-methylumbelliferone + alpha-D-glucopyranose	-	?

Subunits	Comment	Organism
More	building structural models of complexes of the catalytic domain of the enzyme with the imino sugars bound to its active site by homology modeling, and examination of the molecular interaction between them. The active-site pocket is composed of residues D404, I441, W481, W516, D518, M519, R600, D616, F649, and H674	Homo sapiens

Ki Value [mM]	Ki Value maximum [mM]	Inhibitor	Comment	Organism
0.00055	-	1-deoxynojirimycin	the Ki value is calculated	Homo sapiens
0.00236	-	N-methyl-deoxynojirimycin	the Ki value is calculated	Homo sapiens
0.00474	-	N-butyl-deoxynojirimycin	the Ki value is calculated	Homo sapiens
0.0062	-	N-ethyl-deoxynojirimycin	the Ki value is calculated	Homo sapiens