Pathology Alpha-galactosidase

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1 pathology

1.1 agalsidase alpha
1.2 agalsidase beta
1.3 over-the-counter brand names





pathology

a variety of mutations in gene affect synthesis, processing, , stability of enzyme, causes fabry disease, rare lysosomal storage disorder , sphingolipidosis results failure catabolize alpha-d-galactosyl glycolipid moieties.


two enzyme replacement therapies available functionally compensate alpha-galactosidase deficiency. agalsidase alpha , beta both recombinant forms of human α-galactosidase enzyme , both have same amino acid sequence native enzyme. agalsidase alpha , beta differ in structures of oligosaccharide side chains.


agalsidase alpha

the pharmaceutical company shire manufactures agalsidase alfa (inn) under trade name replagal treatment fabry disease, , granted marketing approval in eu in 2001. fda approval applied united states. however, in 2012, shire withdrew application approval in united states citing agency require additional clinical trials before approval.


agalsidase beta

the pharmaceutical company genzyme produces synthetic agalsidase beta (inn) under trade name fabrazyme treatment of fabry disease. in 2009, contamination @ genzyme s allston, massachusetts plant caused worldwide shortage of fabrazyme, , supplies rationed patients @ one-third recommended dose. patients have petitioned break company s patent on drug under march-in provisions of bayh–dole act.


over-the-counter brand names

alpha-galactosidase active ingredient in beano, cvs beanaid, enzymedica s beanassist. these products marketed reduce stomach gas production after eating foods known cause gas. there dozens of generic brands containing enzyme in united states. optimally active @ 55 degrees c, after half-life 120 minutes.








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