高雪氏症的治療藥物

Ceredase® (alglucerase injection) is a modified form of the enzyme, β-glucocerebrosidase (β-Dglucosyl-N-acylsphingosine glucohydrolase, EC 3.2.1.45). Alglucerase is a monomeric glycoprotein of 497 amino acids withcarbohydrates making up approximately 6% of the molecule (Mr = 59,300 as determined by SDS-PAGE). The unmodified enzyme (β-glucocerebrosidase) also contains 497 amino acids and contains approximately 12% carbohydrate (Mr = 67,000). The carbohydrates on the unmodified enzyme consist of N-linked carbohydrate chains of the complex and high mannose type. Glucocerebrosidase and alglucerase catalyze the hydrolysis of the glycolipid, glucocerebroside, within the lysosomes of the reticuloendothelial system.


Cerezyme® (imiglucerase for injection) is an analogue of the human enzyme

ß-glucocerebrosidase, produced by recombinant DNA technology.

ß-Glucocerebrosidase (ß-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 

還記得高雪氏症嗎?

http://mulicia.pixnet.net/blog/post/10593915

最近有一個新藥上市:Velaglucerase alfa for injection

VPRIV.jpg 

 indicated for long-term enzyme replacement therapy in patients with type 1 Gaucher disease.

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