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Target
Lysosomal acid/Non-lysosomal glucosylceramidase
Ligand
BDBM18355
Substrate
n/a
Meas. Tech.
ChEMBL_1672828 (CHEMBL4022857)
IC50
180000±n/a nM
Citation
 D'Alonzo, D; De Fenza, M; Porto, C; Iacono, R; Huebecker, M; Cobucci-Ponzano, B; Priestman, DA; Platt, F; Parenti, G; Moracci, M; Palumbo, G; Guaragna, A N-Butyl-l-deoxynojirimycin (l-NBDNJ): Synthesis of an Allosteric Enhancer ofα-Glucosidase Activity for the Treatment of Pompe Disease. J Med Chem 60:9462-9469 (2017) [PubMed]  Article 
Target
Name:
Lysosomal acid/Non-lysosomal glucosylceramidase
Synonyms:
Glucocerebrosidase GBA1/GBA2
Type:
n/a
Mol. Mass.:
n/a
Description:
ASSAY_ID of ChEMBL is 1672828
Components:
This complex has 2 components.
Component 1
Name:
Lysosomal acid glucosylceramidase
Synonyms:
Acid beta-glucosidase | Alglucerase | Beta-glucocerebrosidase | Beta-glucocerebrosidase (GC) | D-glucosyl-N-acylsphingosine glucohydrolase | GCase | Glucocerebrosidase (GBA) | Glucosylceramidase (GBA) | Glucosylceramidase (GCase) | Glucosylceramidase precursor (Beta-glucocerebrosidase) (Acid beta-glucosidase) (D-glucosyl-N-acylsphingosine glucohydrolase) (Alglucerase) (Imiglucerase) | Imiglucerase | beta-glucocerebrosidase (GCase) | GBA1_HUMAN | GBA1 | GBA | GC | GLUC
Type:
Enzyme
Mol. Mass.:
59724.64
Organism:
Human
Description:
The beta-Glu activity was measured with commercially available beta-glucocerebrosidase (Ceredase) as the enzyme source.
Residue:
536
Sequence:
MEFSSPSREECPKPLSRVSIMAGSLTGLLLLQAVSWASGARPCIPKSFGYSSVVCVCNATYCDSFDPPTFPALGTFSRYESTRSGRRMELSMGPIQANHTGTGLLLTLQPEQKFQKVKGFGGAMTDAAALNILALSPPAQNLLLKSYFSEEGIGYNIIRVPMASCDFSIRTYTYADTPDDFQLHNFSLPEEDTKLKIPLIHRALQLAQRPVSLLASPWTSPTWLKTNGAVNGKGSLKGQPGDIYHQTWARYFVKFLDAYAEHKLQFWAVTAENEPSAGLLSGYPFQCLGFTPEHQRDFIARDLGPTLANSTHHNVRLLMLDDQRLLLPHWAKVVLTDPEAAKYVHGIAVHWYLDFLAPAKATLGETHRLFPNTMLFASEACVGSKFWEQSVRLGSWDRGMQYSHSIITNLLYHVVGWTDWNLALNPEGGPNWVRNFVDSPIIVDITKDTFYKQPMFYHLGHFSKFIPEGSQRVGLVASQKNDLDAVALMHPDGSAVVVVLNRSSKDVPLTIKDPAVGFLETISPGYSIHTYLWRRQ
  
Component 2
Name:
Non-lysosomal glucosylceramidase
Synonyms:
GBA2_HUMAN | GBA2 | KIAA1605 | SPG46 | Beta-glucosidase
Type:
PROTEIN
Mol. Mass.:
104639.04
Organism:
Human
Description:
ChEMBL_1435476
Residue:
927
Sequence:
MGTQDPGNMGTGVPASEQISCAKEDPQVYCPEETGGTKDVQVTDCKSPEDSRPPKETDCCNPEDSGQLMVSYEGKAMGYQVPPFGWRICLAHEFTEKRKPFQANNVSLSNMIKHIGMGLRYLQWWYRKTHVEKKTPFIDMINSVPLRQIYGCPLGGIGGGTITRGWRGQFCRWQLNPGMYQHRTVIADQFTVCLRREGQTVYQQVLSLERPSVLRSWNWGLCGYFAFYHALYPRAWTVYQLPGQNVTLTCRQITPILPHDYQDSSLPVGVFVWDVENEGDEALDVSIMFSMRNGLGGGDDAPGGLWNEPFCLERSGETVRGLLLHHPTLPNPYTMAVAARVTAATTVTHITAFDPDSTGQQVWQDLLQDGQLDSPTGQSTPTQKGVGIAGAVCVSSKLRPRGQCRLEFSLAWDMPRIMFGAKGQVHYRRYTRFFGQDGDAAPALSHYALCRYAEWEERISAWQSPVLDDRSLPAWYKSALFNELYFLADGGTVWLEVLEDSLPEELGRNMCHLRPTLRDYGRFGYLEGQEYRMYNTYDVHFYASFALIMLWPKLELSLQYDMALATLREDLTRRRYLMSGVMAPVKRRNVIPHDIGDPDDEPWLRVNAYLIHDTADWKDLNLKFVLQVYRDYYLTGDQNFLKDMWPVCLAVMESEMKFDKDHDGLIENGGYADQTYDGWVTTGPSAYCGGLWLAAVAVMVQMAALCGAQDIQDKFSSILSRGQEAYERLLWNGRYYNYDSSSRPQSRSVMSDQCAGQWFLKACGLGEGDTEVFPTQHVVRALQTIFELNVQAFAGGAMGAVNGMQPHGVPDKSSVQSDEVWVGVVYGLAATMIQEGLTWEGFQTAEGCYRTVWERLGLAFQTPEAYCQQRVFRSLAYMRPLSIWAMQLALQQQQHKKASWPKVKQGTGLRTGPMFGPKEAMANLSPE
  
Inhibitor
Name:
BDBM18355
Synonyms:
N-Butyl-DNJ | (2R,3R,4R,5S)-1-butyl-2-(hydroxymethyl)piperidine-3,4,5-triol | CHEMBL1029 | MIGLUSTAT | US9181184, 5 | US20230339856, Compound NB-DNJ
Type:
Small organic molecule
Emp. Form.:
C10H21NO4
Mol. Mass.:
219.15
SMILES:
CCCCN1C[C@H](O)[C@@H](O)[C@H](O)[C@H]1CO
Structure:
Search PDB for entries with ligand similarity: