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Review of the Medical Use of Agalsidase Beta (Fabrazyme)

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Review of the Medical Use of Agalsidase Beta (Fabrazyme)

4.85 84,593 Ratings
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Editor's Note:
Fabrazyme(R) (agalsidase beta) is a recombinant human alpha-galactosidase A enzyme with the same amino acid sequence as the native enzyme. Purified agalsidase beta is a homodimeric glycoprotein with a molecular weight of approximately 100 kD. The mature protein is comprised of two subunits of 398 amino acids (approximately 51 kD), each of which contains three N-linked glycosylation sites. alpha-galactosidase A catalyzes the hydrolysis of globotriaosylceramide (GL-3) and other alpha-galactyl-terminated neutral glycosphingolipids, such as galabiosylceramide and blood group B substances to ceramide dihexoside and galactose. The specific activity of Fabrazyme (agalsidase beta) is approximately 70 U/mg (one unit is defined as the amount of activity that results in the hydrolysis of 1 rjmoie of a synthetic substrate, p-nitrophenyl-alpha-D-galactopyranoside, per minute under the assay conditions). Fabrazyme (agalsidase beta) is produced by recombinant DNA technology in a Chinese Hamster Ovary mammalian cell expression system. Fabrazyme (agalsidase beta) is intended for intravenous infusion. It is supplied as a sterile, nonpyrogenic, white to off-white, lyophilized cake or powder for reconstitution with Sterile Water for Injection, uSp. Each 35 mg vial contains 37 mg of agalsidase beta as well as 222 mg mannitol, 20.4 mg sodium phosphate monobasic monohydrate, and 59.2 mg sodium phosphate dibasic heptahydrate. Following reconstitution as directed, 35 mg of agalsidase beta (7 mL) may be extracted from each 35 mg vial. Each 5 mg vial contains 5.5 mg of agalsidase beta as well as 33.0 mg mannitol, 3.0 mg sodium phosphate monobasic monohydrate, and 8.8 mg sodium phosphate dibasic heptahydrate. Following reconstitution as directed, 5 mg of agalsidase beta (1 mL) may be extracted from each 5 mg vial.

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Length 86 Pages
Imprint Createspace Independent Publishing Platform
Released N/A
Identifier (ISBN) 43273085
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After spending years working in the corporate world, Xavier Research Press finally decided to pursue a lifelong passion for writing. They currently reside in a quiet suburb where they spend most of their time outlining new stories, reading extensively, and occasionally attempting to bake.

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Joseph Thomas
Joseph Thomas October 10, 2024
"Exceeded all my expectations. The prose is exquisite. It’s accessible yet undeniably sophisticated. I will undoubtedly revisit this masterpiece."
Daniel Scott
Daniel Scott October 25, 2024
"I find myself somewhat conflicted. The tempo was somewhat languid, and I found my mind wandering. Not poor, simply average. Perfectly average."
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Michelle Edwards
Michelle Edwards August 27, 2025
"Took a moment to build, but the payoff was spectacular. The middle had a deliberate pace, but the climax was phenomenal. A very distinguished novel."
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Andrew Allen January 26, 2025
"Masterpiece from start to finish. The prose is exquisite. It’s accessible yet undeniably sophisticated. An effortless 5 stars."
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Linda White February 16, 2025
"Honestly one of the most premium reading experiences lately. The author orchestrated a breathtaking finale. Truly a work of art. A true literary gem."
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Sharon Campbell February 20, 2025
"Such a rich narrative. It strikes a perfect harmony of dialogue and world-building. Every chapter felt necessary. A true literary gem."
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Eric Collins
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"Not entirely to my refined tastes. It verged on repetitive in the second act. A more concise edit would have served it well. Perhaps more aligned with a different audience's tastes."
Jacob Perez
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"A decent, if standard, experience. It possessed moments of brilliance, but the narrative felt slightly disjointed ultimately. Perhaps more aligned with a different audience's tastes."
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"I thoroughly enjoyed the elegance of it. It held my rapt attention, even when certain twists were telegraphed. I would highly recommend this to discerning readers."
Thomas Ramirez
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"A very strong entry. It held my rapt attention, even when certain twists were telegraphed. Quite impressive overall."
Amanda Edwards
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"Absolutely brilliant. It strikes a perfect harmony of dialogue and world-building. Every chapter felt necessary. Worth every moment spent reading."
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"Absolutely brilliant. The prose is exquisite. It’s accessible yet undeniably sophisticated. An absolute must-read for connoisseurs of the genre."
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George Walker
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"A highly sophisticated read. Beautiful writing, though I craved slightly more depth in the lore. Still, undeniably engaging. A wonderful companion for a quiet weekend."
Matthew Miller
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"Took a moment to build, but the payoff was spectacular. Beautiful writing, though I craved slightly more depth in the lore. Still, undeniably engaging. A wonderful companion for a quiet weekend."
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Deborah Gomez September 10, 2024
"Such a rich narrative. The prose is exquisite. It’s accessible yet undeniably sophisticated. I will undoubtedly revisit this masterpiece."
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"Exceeded all my expectations. The author orchestrated a breathtaking finale. Truly a work of art. Worth every moment spent reading."
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"A highly sophisticated read. It held my rapt attention, even when certain twists were telegraphed. I would highly recommend this to discerning readers."
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Barbara Collins May 28, 2024
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Susan Rivera July 12, 2024
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"Masterpiece from start to finish. The author orchestrated a breathtaking finale. Truly a work of art. I am now a lifelong fan of this author."
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"A very strong entry. The middle had a deliberate pace, but the climax was phenomenal. A very distinguished novel."
Anthony Wright
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"Exceeded all my expectations. The author orchestrated a breathtaking finale. Truly a work of art. Worth every moment spent reading."
Patricia Smith
Patricia Smith February 21, 2024
"A highly sophisticated read. It held my rapt attention, even when certain twists were telegraphed. Quite impressive overall."
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"I thoroughly enjoyed the elegance of it. A brilliant concept executed with flair. Just a few pacing choices I would change. Quite impressive overall."
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"Such a rich narrative. The pacing was impeccable and the characters felt incredibly authentic. I was completely immersed. A true literary gem."
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"Honestly one of the most premium reading experiences lately. The pacing was impeccable and the characters felt incredibly authentic. I was completely immersed. A true literary gem."
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"A very strong entry. The middle had a deliberate pace, but the climax was phenomenal. A wonderful companion for a quiet weekend."
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"Absolutely brilliant. The pacing was impeccable and the characters felt incredibly authentic. I was completely immersed. An effortless 5 stars."
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"A profoundly satisfying read. I became deeply invested in the protagonist's journey. A masterclass in storytelling. An effortless 5 stars."
Susan Lewis
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"Absolutely brilliant. It strikes a perfect harmony of dialogue and world-building. Every chapter felt necessary. An effortless 5 stars."
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"Took a moment to build, but the payoff was spectacular. The middle had a deliberate pace, but the climax was phenomenal. A wonderful companion for a quiet weekend."
Kenneth Sanchez
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"I thoroughly enjoyed the elegance of it. It held my rapt attention, even when certain twists were telegraphed. I would highly recommend this to discerning readers."
Donald Adams
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Jennifer Lopez
Jennifer Lopez March 10, 2025
"I was completely blown away. The author orchestrated a breathtaking finale. Truly a work of art. Worth every moment spent reading."
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"Exceeded all my expectations. The pacing was impeccable and the characters felt incredibly authentic. I was completely immersed. I am now a lifelong fan of this author."
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"I was completely blown away. The pacing was impeccable and the characters felt incredibly authentic. I was completely immersed. I am now a lifelong fan of this author."
Donna Nelson
Donna Nelson May 18, 2024
"Exceeded all my expectations. I adored how the story unfolded. It kept me captivated without sacrificing elegance. I will undoubtedly revisit this masterpiece."
Lisa Williams
Lisa Williams January 25, 2024
"I was completely blown away. The pacing was impeccable and the characters felt incredibly authentic. I was completely immersed. An absolute must-read for connoisseurs of the genre."
Eric Wilson
Eric Wilson April 03, 2024
"I thoroughly enjoyed the elegance of it. The protagonist was compelling, though some subplots felt slightly overshadowed. A very distinguished novel."
Melissa Green
Melissa Green April 21, 2025
"Such a rich narrative. I became deeply invested in the protagonist's journey. A masterclass in storytelling. An absolute must-read for connoisseurs of the genre."
Nicholas Young
Nicholas Young May 23, 2024
"It was adequate. The tempo was somewhat languid, and I found my mind wandering. Not poor, simply average. A fair 3 stars."
Ryan Phillips
Ryan Phillips August 18, 2024
"Absolutely brilliant. The pacing was impeccable and the characters felt incredibly authentic. I was completely immersed. I am now a lifelong fan of this author."
Deborah Gomez
Deborah Gomez September 04, 2024
"Absolutely brilliant. I became deeply invested in the protagonist's journey. A masterclass in storytelling. An absolute must-read for connoisseurs of the genre."
Charles Green
Charles Green May 22, 2024
"I was completely blown away. The author orchestrated a breathtaking finale. Truly a work of art. An absolute must-read for connoisseurs of the genre."
Richard Robinson
Richard Robinson April 14, 2025
"I find myself somewhat conflicted. Certain acts were fascinating, while others felt like a slight endeavor to complete. Perhaps more aligned with a different audience's tastes."
Paul Gonzalez
Paul Gonzalez January 30, 2025
"Masterpiece from start to finish. I became deeply invested in the protagonist's journey. A masterclass in storytelling. An effortless 5 stars."
Charles Jackson
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"Honestly one of the most premium reading experiences lately. The pacing was impeccable and the characters felt incredibly authentic. I was completely immersed. An absolute must-read for connoisseurs of the genre."
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