A competitive inhibitor of human lysosomal β-glucosidase (Ki = 0.016-0.025 µM; IC50 = 0.06 µM) that interacts with the catalytic pocket of β-glucosidase

Isofagomine (D-tartrate) – 5 mg

Brand:
Cayman
CAS:
957230-65-8
Storage:
-20
UN-No:
Non-Hazardous - /

Isofagomine (D-tartrate) is a competitive inhibitor of human lysosomal β-glucosidase (Ki = 0.016-0.025 µM; IC50 = 0.06 µM).{22626} By interacting with the catalytic pocket of β-glucosidase it acts as a chemical chaperone that increases the amount of β-glucosidase by stabilizing and/or promoting the folding of the enzyme.{25308} Isofagomine (D-tartrate) has been shown to increase lysosomal β-glucosidase activity by 2- to 3-fold in mutant N370S Gaucher fibroblasts.{22626} This compound has been studied in the context of Gaucher disease, a lysosomal storage disorder resulting from substantial deficiency of β-glucosidase and recently identified as a parkinsonism risk factor.{25308}  

 

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Description

A competitive inhibitor of human lysosomal β-glucosidase (Ki = 0.016-0.025 µM; IC50 = 0.06 µM) that interacts with the catalytic pocket of β-glucosidase, acting as a chemical chaperone that stabilizes and/or promotes the folding of the enzyme; increases lysosomal β-glucosidase activity by 2- to 3-fold in mutant N370S Gaucher fibroblasts

Formal name: 5R-(hydroxymethyl)-3R,4R-piperidinediol, mono 2S,3S-dihydroxybutanedioate

Synonyms: 

Molecular weight: 297.3

CAS: 957230-65-8

Purity: ≥95%

Formulation: A solution in ethanol:water (1:1)

Product Type|Biochemicals|Small Molecule Inhibitors||Research Area|Endocrinology & Metabolism|Inborn Errors of Metabolism||Research Area|Neuroscience|Neurodegenerative Disorders|Parkinson’s Disease

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