Target
Lysosomal acid glucosylceramidase
Ligand
BDBM108217
Substrate
n/a
Meas. Tech.
GCase IC50 Assay
pH
5.2±0
IC50
5.34±0.0 nM
Citation
 Li, ZLi, TDai, SXie, XMa, XZhao, WZhang, WLi, JWang, PG New insights into the pharmacological chaperone activity of c2-substituted glucoimidazoles for the treatment of Gaucher disease. Chembiochem 14:1239-47 (2013) [PubMed]  Article 
Target
Name:
Lysosomal acid glucosylceramidase
Synonyms:
Acid beta-glucosidase | Alglucerase | Beta-glucocerebrosidase | Beta-glucocerebrosidase (GC) | D-glucosyl-N-acylsphingosine glucohydrolase | GBA | GBA1 | GBA1_HUMAN | GC | GCase | GLUC | Glucocerebrosidase (GBA) | Glucosylceramidase (GBA) | Glucosylceramidase (GCase) | Glucosylceramidase precursor (Beta-glucocerebrosidase) (Acid beta-glucosidase) (D-glucosyl-N-acylsphingosine glucohydrolase) (Alglucerase) (Imiglucerase) | Imiglucerase | beta-glucocerebrosidase (GCase)
Type:
Enzyme
Mol. Mass.:
59724.64
Organism:
Homo sapiens (Human)
Description:
The beta-Glu activity was measured with commercially available beta-glucocerebrosidase (Ceredase) as the enzyme source.
Residue:
536
Sequence:
MEFSSPSREECPKPLSRVSIMAGSLTGLLLLQAVSWASGARPCIPKSFGYSSVVCVCNATYCDSFDPPTFPALGTFSRYESTRSGRRMELSMGPIQANHTGTGLLLTLQPEQKFQKVKGFGGAMTDAAALNILALSPPAQNLLLKSYFSEEGIGYNIIRVPMASCDFSIRTYTYADTPDDFQLHNFSLPEEDTKLKIPLIHRALQLAQRPVSLLASPWTSPTWLKTNGAVNGKGSLKGQPGDIYHQTWARYFVKFLDAYAEHKLQFWAVTAENEPSAGLLSGYPFQCLGFTPEHQRDFIARDLGPTLANSTHHNVRLLMLDDQRLLLPHWAKVVLTDPEAAKYVHGIAVHWYLDFLAPAKATLGETHRLFPNTMLFASEACVGSKFWEQSVRLGSWDRGMQYSHSIITNLLYHVVGWTDWNLALNPEGGPNWVRNFVDSPIIVDITKDTFYKQPMFYHLGHFSKFIPEGSQRVGLVASQKNDLDAVALMHPDGSAVVVVLNRSSKDVPLTIKDPAVGFLETISPGYSIHTYLWRRQ
  
Inhibitor
Name:
BDBM108217
Synonyms:
2-(3,3-dimethylbutyl)-5-(hydroxymethyl)- 5H,6H,7H,8H-imidazo[1,2-a]pyridine-6,7,8-triol (Compound 3)
Type:
Small organic molecule
Emp. Form.:
C14H24N2O4
Mol. Mass.:
284.3514
SMILES:
CC(C)(C)CCc1cn2C(CO)C(O)C(O)C(O)c2n1
Structure:
Search PDB for entries with ligand similarity: