Specification
Description
Imiglucerase is a hydrolytic lysosomal glucocerebrosidase-specific enzyme. It is ananalogue of the human enzyme b-glucocerebrosidase (b-D-glucosyl-N-acylsphingosineglucohydrolase, E.C. 3.2.1.45), produced by recombinant DNA technology usingmammalian cell culture (Chinese hamster ovary). Purified imiglucerase is a monomericglycoprotein of 497 amino acids, containing 4 N-linked glycosylation sites (Mr=60,430).Imiglucerase differs from placental glucocerebrosidase by one amino acid at position495, where histidine is substituted for arginine
Storage
Store at -20°C to -80°C
Formulation
Each vial contains 42.4 units imiglucerase, mannitol (34 mg), polysorbate 80, NF (0.106mg), and sodium citrates: disodium hydrogen citrate (3.6 mg) and trisodium citrate (10.4mg).
Unit Definition
An enzyme unit (U) is defined as the amount of enzyme that catalyzes the hydrolysis of1 micromole of the synthetic substrate para-nitrophenyl-b-D-glucopyranoside (pNP-Glc)per minute at 37°C. Reconstituted solutions have a pH of approximately 6.1