Glucosylcerebrosidase

Glucosylcerebrosidase Uses, Dosage, Side Effects, Food Interaction and all others data.

Glucosylcerebrosidase is the recombinant active form of the human lysosomal enzyme, β-glucocerebrosidase. It was approved in 2012 and is marketed under the name Elelyso for use in patients with type 1 Gaucher's disease.

Patient's with Type 1 Gaucher disease have a long-term deficiency in the enzyme, glucocerebrosidase. Glucosylcerebrosidase is a modified form of glucocerebrosidase and is provided to counter this enzyme deficiency, resulting in smaller liver and spleen size, and improved thrombocytopenia and anemia.

Trade Name Glucosylcerebrosidase
Generic Taliglucerase alfa
Taliglucerase alfa Other Names Glucosylcerebrosidase, prGC-D, prGCD, Taliglucerase alfa
Type
Formula C2580H3918N680O727S17
Weight 56637.9397 Da
Protein binding

Plasma protein binding was not quantified.

Groups Approved, Investigational
Therapeutic Class
Manufacturer
Available Country
Last Updated: September 19, 2023 at 7:00 am
Glucosylcerebrosidase
Glucosylcerebrosidase

Uses

Glucosylcerebrosidase is a hydrolytic lysosomal glucocerebroside specific enzyme, used for long term enzyme replacement therapy in patients with Type 1 Gaucher disease.

For the treatment of adult Type 1 Gaucher disease.

Glucosylcerebrosidase is also used to associated treatment for these conditions: Gaucher Disease, Type 1, Type 3 Gaucher disease

How Glucosylcerebrosidase works

Glucosylcerebrosidase is different from human glucocerebrosidase by two amino acids at the N terminal and up to 7 amino acids at the C terminal. This recombinant enzyme allows the hydrolysis reaction of glucocerebroside to glucose and ceramide that naturally occurs in healthy individuals.

Toxicity

The most common toxic reaction seen was infusion reactions such as urticaria, arthralgia, headache, and chest pain due to IV administration.

Food Interaction

  • Take with or without food.

Volume of Distribution

The steady state volume of distribution is between 7.30 to 11.7 L.

Elimination Route

Glucosylcerebrosidase is administered IV so absorption is 100%.

Half Life

The half life is between 18.9 to 28.7 min.

Clearance

The systemic clearance was approximately 30 L/hr and 20 L/hr for 30 and 60 units/kg, respectively.

Elimination Route

Route of elimination was not determined.

Innovators Monograph

You find simplified version here Glucosylcerebrosidase

*** Taking medicines without doctor's advice can cause long-term problems.
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