Agalsidase Beta for Injection.

Drug Name]:
Agalsidase β for injection
[Approval No.]:
S20190040
[Ingredients]:
The active ingredient is agalsidase β. Chemical name: recombinant human α-galactosidase A, referred to as rhαGAL. Molecular formula (based on amino acid sequence): C2029H3080N5440587S27 Agalsidase β is a recombinant human α-galactosidase A, and its amino acid sequence is the same as the natural enzyme. Purified agalsidase β is a homodimeric glycoprotein with a molecular weight of about 100kD. The mature protein consists of 2 subunits of 398 amino acids (about 51kD), each of which contains 3 N-linked glycosylation sites. Excipients: mannitol, sodium dihydrogen phosphate monohydrate, sodium dihydrogen phosphate heptahydrate.
[Properties]:
White or off-white freeze-dried block or powder.
This product should be used under the supervision of a physician who has experience in managing patients with Fabry disease or other inherited metabolic diseases.

Dosage

The recommended dose of this product is 1 mg/kg, administered by intravenous infusion once every 2 weeks.

The initial infusion rate should not exceed 0.25 mg/min (15 mg/hr) to minimize the occurrence of infusion-related reactions….
Because agalsidase beta (r-hagal) is a recombinant protein, IgG antibodies are expected to appear in patients with low or no residual enzyme activity. Most patients generally develop IgG antibodies to r-haGAL within 3 months after the first infusion of agalsidase beta for injection. Over time, most serum reactions in clinical trials….