A modified form of beta-glucerebrosidase that catalyses the hydrolysis of glucocerebroside in reticuloendothelial system lysosomes, which is the most effective therapy for type-1 Gaucher disease, resulting in decreased hepatospenomegaly, haematologic defects, increased bone mineralisation and reversal of cachexia seen in these patients.


 A recombinant glucocerebrosidase used for Gaucher's disease
References in periodicals archive ?
Specific therapy for the nonneuronopathic manifestations of Gaucher disease has been available since 1991 firstly in the form of the macrophage targeted placenta-derived glucocerebrosidase (alglucerase, Ceredase [R], Genzyme Corporation, MA) (7), and subsequently (1994 in USA and 1997 in Europe) by recombinant human enzyme, imiglucerase (Cerezyme[R], Genzyme Corporation, MA) (8).
When Congress called Genzyme to task for profiteering, the company's CEO, Henri Termeer, fired back in the pages of The Wall Street Journal that the company had every right to charge what the market would bear, because it had risked everything to develop Ceredase.
For Brian, one of only about 5,000 Gaucher disease patients in the world, the introduction of Ceredase was truly a miracle.
The only medicine currently available to treat it, called Ceredase, costs an average of $160,000 a year, said Cramer.
A woman suffering from Gaucher's disease, a rare life-threatening illness that weakens bones and swells organs, told the press that it costs her $350,000 a year to buy Ceredase, the drug that keeps her alive.
Ceredase, a drug for treating a rare enzyme deficiency called Gaucher's disease, now cost an average of $140,000 a year.
The nonrecombinant products ceredase and adenosine deaminase (PEG-ADA) illustrate the potential impact of chronic lifetime administration of drugs.
Aliski also brings a wealth of relevant commercial experience from his eight years at Genzyme Corporation where he was primarily responsible for reimbursement, distribution and patient support services for the Ceredase and Cerezyme, the first enzyme replacement therapy to treat Gaucher Disease.
Operating profit margins in the quarter were impacted by a change in the overall business mix, which included a higher percentage of revenue generated from Ceredase and Synagis programs.
Genzyme launched its first tissue-derived enzyme replacement product, Ceredase, in April 1991, to treat Gaucher disease, after conducting a single clinical trial.
Murphy, president and chief executive officer of Bentley, said, "We are particularly pleased with the continuing sales growth and improved gross margins in our Spanish operations, which have partially offset the decrease in sales volume and low gross margins associated with the March 31, 1996 expiration of the Ceredase marketing agreement in France.
A distribution agreement for the product Ceredase, which accounted for approximately 60 percent of the company's revenues in 1995 and approximately 54 percent of its revenues in the first quarter of 1996, expired on March 31, 1996.