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
Fabrazyme Online, Description, Chemistry, Ingredients - Agalsidase Beta
Fabrazyme Online, Description, Chemistry, Ingredients - Agalsidase Beta
DESCRIPTION
FabrazymeÒ is
a recombinant human a-galactosidase A enzyme with the same amino acid sequence
as the native enzyme. Purified agalsidase beta is a homodimeric glycoprotein
with a molecular weight of approximately 100 KD. The mature protein is comprised
of two subunits of 398 amino acids (approximately 51 KD), each of which contains
three N-linked glycosylation sites. a-galactosidase
A catalyzes the hydrolysis of globotriaosy-lceramide (GL-3) and other a-galactyl-terminated
neutral glycosphingolipids, such as galabiosylceramide and blood group B substances
to ceramide dihexoside and galactose. The specific activity of Fabrazyme®
is approximately 70 U/mg (one unit is defined as the amount of activity that
results in the hydrolysis of 1 µmole of a synthetic substrate, p-nitrophenyl-a-D-galactopyranoside,
per minute under the assay conditions).
FabrazymeÒ is
produced by recombinant DNA technology in a Chinese Hamster Ovary mammalian
cell expression system.
FabrazymeÒ is
intended for intravenous infusion. It is supplied as a sterile, nonpyrogenic,
white to off-white, lyophilized cake or powder for reconstitution with Sterile
Water for Injection, USP. Each vial contains 37 mg of agalsidase beta as well
as 222 mg mannitol, 20.4 mg sodium phosphate monobasic monohydrate, and 59.2
mg sodium phosphate dibasic heptahydrate. Following reconstitution as directed,
35 mg of agalsidase beta (7 mL) may be extracted from each vial.
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