Tìm theo
Agalsidase beta
Các tên gọi khác (5 ) :
  • Agalsidase alfa
  • Alpha-D- galactoside galactohydrolase
  • Alpha-D-galactosidase A
  • Alpha-galactosidase A precursor
  • Melibiase
enzyme replacement agents
Thuốc Gốc
Biotech
CAS: 104138-64-9
ATC: A16AB04
ĐG : Genzyme Inc. , http://www.genzyme.ca
CTHH: C2029H3080N544O587S27
PTK: 45351.6000
Recombinant human alpha-galactosidase A. The mature protein is composed of 2 subunits of 398 residues. Protein is glycosylated and produced by CHO cells
Nhận Dạng Quốc Tế & Đặc Tính Hóa Học
Công thức hóa học
C2029H3080N544O587S27
Phân tử khối
45351.6000
Độ kỵ nước
-0.307
Điểm đẳng điện tích
5.17
Dược Lực Học : Used in the treatment of Fabry disease, an X-linked genetic disorder of glycosphingolipid metabolism. The disease is characterized by a deficiency of the lysosomal enzyme alpha-galactosidase A, which leads to progressive accumulation of glycosphingolipids, predominantly GL-3, in many body tissues. Clinical manifestations of Fabry disease include renal failure, cardiomyopathy, and cerebrovascular accidents. Fabrazyme is intended to provide an exogenous source of alpha-galactosidase A and to limit the accumulation of these glycolipids in the tissues. Studies show unconclusive evidence for the clinical efficacy of either agalasidase alfa or agalasidase beta in preventing morbidity.
Cơ Chế Tác Dụng : Recombinant human alpha-galactosidase A. The mature protein is composed of 2 subunits of 398 residues. Protein is glycosylated and produced by CHO cells Alpha-galactosidase A catalyzes the hydrolysis of globotriaosylceramide (GL-3) and other a-galactyl-terminated neutral glycosphingolipids, such as galabiosylceramide and blood group B substances to ceramide dihexoside and galactose.
Dược Động Học :

▧ Half Life :
45-102 min
▧ Clearance :
* 4.1 +/- 1.2 mL/min/kg [adult patients with Fabry disease,0.3 mg/kg, 1 infusion] * 4.6 +/- 2.2 mL/min/kg [adult patients with Fabry disease, 0.3 mg/kg, 5 infusions] * 2.1 +/- 0.7 mL/min/kg [adult patients with Fabry disease, 1 mg/kg, 1 infusion] * 3.2 +/- 2.6 mL/min/kg [adult patients with Fabry disease, 1 mg/kg, 5 infusions] * 0.8 +/- 0.3 mL/min/kg [adult patients with Fabry disease, 3 mg/kg, 1 infusion] * 0.8 +/- 0.4 mL/min/kg [adult patients with Fabry disease, 3 mg/kg, 5 infusions] * 1.8 +/- 0.8 mL/min/kg [Pediatric Patients with Fabry Disease, 1 mg/kg, 1-3 infusions] * 4.9 +/- 5.6 mL/min/kg [Pediatric Patients with Fabry Disease, 1 mg/kg, 7 infusions] * 2.3 +/- 2.2 mL/min/kg [Pediatric Patients with Fabry Disease, 1 mg/kg, 11 infusions]
Chỉ Định : For treatment of Fabry's disease (alpha-galactosidase A deficiency)
Liều Lượng & Cách Dùng : Powder, for solution - Intravenous
Dữ Kiện Thương Mại
Giá thị trường
  • Biệt dược thương mại : Fabrazyme 5 mg vial
    Giá bán buôn : USD >771.6
    Đơn vị tính : vial
  • Biệt dược thương mại : Fabrazyme 35 mg vial
    Giá bán buôn : USD >5403.6
    Đơn vị tính : vial
Nhà Sản Xuất
  • Công ty : Genzyme Corp
    Sản phẩm biệt dược : Fabrazyme
  • Công ty :
    Sản phẩm biệt dược : Replagal
Đóng gói
Tài Liệu Tham Khảo Thêm
drugbank
http://www.drugbank.ca/drugs/DB00103
National Drug Code Directory
http://www.hipaaspace.com/Medical_Billing/Coding/National.Drug.Codes/PDF/58468-0040-1
GenBank
http://www.ncbi.nlm.nih.gov/nuccore/X14448
PharmGKB
http://www.pharmgkb.org/drug/PA164746527
Wikipedia
http://en.wikipedia.org/wiki/Agalsidase_beta
RxList
http://www.rxlist.com/cgi/generic3/fabrazyme.htm
Drugs.com
http://www.drugs.com/cdi/agalsidase-beta.html
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