A1,
A2,
B,
C1,
C2,
D,
E,
F,
G-H,
I-K,
L,
M,
N,
O,
P1,
P2,
Q-R,
S,
T,
U-V,
W-Z
Cerezyme Online, Description, Chemistry, Ingredients - Imiglucerase
Cerezyme Online, Description, Chemistry, Ingredients - Imiglucerase
DESCRIPTION
Cerezyme ® (imiglucerase for injection) is an analogue of the human
enzyme, (beta)-glucocerebrosidase produced by recombinant DNA technology. (beta)-Glucocerebrosidase
((beta)-D-glucosyl-N-acylsphingosine glucohydrolase, E.C. 3.2.1.45) is a lysosomal
glycoprotein enzyme which catalyzes the hydrolysis of the glycolipid glucocerebroside
to glucose and ceramide.
Cerezyme ® is produced by recombinant DNA technology using mammalian
cell culture (Chinese hamster ovary). Purified imiglucerase is a monomeric glycoprotein
of 497 amino acids, containing 4 N-linked glycosylation sites (Mr = 60,430).
Imiglucerase differs from placental glucocerebrosidase by one amino acid at
position 495 where histidine is substituted for arginine. The oligosaccharide
chains at the glycosylation sites have been modified to terminate in mannose
sugars. The modified carbohydrate structures on imiglucerase are somewhat different
from those on placental glucocerebrosidase. These mannose-terminated oligosaccharide
chains of imiglucerase are specifically recognized by endocytic carbohydrate
receptors on macrophages, the cells that accumulate lipid in Gaucher disease.
Cerezyme ® is supplied as a sterile, non-pyrogenic, white to off-white
lyophilized product. The quantitative composition of the lyophilized drug is
provided in the following table:
|
Ingredient
|
200 Unit Vial |
400 Unit Vial |
|
Imiglucerase (total amount) *
|
212 units |
424 units |
|
Mannitol
|
170 mg |
340 mg |
|
Sodium Citrates
(Trisodium Citrate)
(Disodium Hydrogen Citrate)
|
70 mg
(52 mg)
(18 mg) |
140 mg
(104 mg)
(36 mg) |
|
Polysorbate 80, NF
|
0.53 mg |
1.06 mg |
|
Citric Acid and/or Sodium Hydroxide may have been added
at the time of manufacture to adjust pH.
* This provides a respective withdrawal dose of 200 and
400 units of imigulcerase.
|
An enzyme unit (U) is defined as the amount of enzyme that catalyzes the hydrolysis
of one micromole of the synthetic substrate para-nitrophenyl-(beta)-D-glucopyranoside
(pNP-Glc) per minute at 37°C. The product is stored at 2-8°C (36-46°F). After
reconstitution with Sterile Water for Injection, USP, the imiglucerase concentration
is 40 U/mL (see DOSAGE AND ADMINISTRATION for final concentrations and
volumes). Reconstituted solutions have a pH of approximately 6.1.
In addition, Haemaccel® (cross-linked gelatin polypeptides), which is used
as a stabilizing agent during the manufacturing process, may also be present
in very small amounts in the final product.
top
