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Laronidase
Các tên gọi khác (1) :
  • Alpha-L-iduronidase precursor
enzyme replacement agents
Thuốc Gốc
Biotech
CAS: 210589-09-6
ATC: A16AB05
ĐG : BioMarin Pharmaceuticals Inc. , http://www.bmrn.com
CTHH: C3160H4848N898O881S12
PTK: 69899.4000
Human recombinant alpha-L-iduronidase, 628 residues (mature form), produced by recombinant DNAtechnology in a Chinese hamster ovary cell line. Laronidase is a glycoprotein with a molecular weight of approximately 83 kD. The predicted amino acid sequence of the recombinant form, as well as the nucleotide sequence that encodes it, are identical to a polymorphic form of human a-L-iduronidase. It contains 6 N-linked oligosaccharide modification sites.
Nhận Dạng Quốc Tế & Đặc Tính Hóa Học
Công thức hóa học
C3160H4848N898O881S12
Phân tử khối
69899.4000
Độ kỵ nước
-0.300
Điểm đẳng điện tích
9.09
Dược Lực Học : Laronidase is used to treat mucopolysaccharide storage disorders (specifically mucopolysaccharidosis 1 or Hurlers syndrome) caused by deficiencies of alpha-L-iduronidase. Reduced or absent a-L-iduronidase activity results in the accumulation of the GAG substrates, dermatan sulfate and heparan sulfate, throughout the body and leads to widespread cellular, tissue, and organ dysfunction.
Cơ Chế Tác Dụng : Human recombinant alpha-L-iduronidase, 628 residues (mature form), produced by recombinant DNAtechnology in a Chinese hamster ovary cell line. Laronidase is a glycoprotein with a molecular weight of approximately 83 kD. The predicted amino acid sequence of the recombinant form, as well as the nucleotide sequence that encodes it, are identical to a polymorphic form of human a-L-iduronidase. It contains 6 N-linked oligosaccharide modification sites. Laronidase catalyses the hydrolysis of terminal alpha-L-iduronic acid residues of dermatan sulfate and heparin sulfate.
Dược Động Học :

▧ Half Life :
1.5-3.6 hrs
Chỉ Định : For the treatment of mucopolysaccharidosis
Liều Lượng & Cách Dùng : Solution - Intravenous
Dữ Kiện Thương Mại
Giá thị trường
Nhà Sản Xuất
Đóng gói
Tài Liệu Tham Khảo Thêm
drugbank
http://www.drugbank.ca/drugs/DB00090
GenBank
http://www.ncbi.nlm.nih.gov/nuccore/M74715
PharmGKB
http://www.pharmgkb.org/drug/PA164776670
Wikipedia
http://en.wikipedia.org/wiki/Laronidase
RxList
http://www.rxlist.com/cgi/generic3/aldurazyme.htm
Drugs.com
http://www.drugs.com/cdi/laronidase.html
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