JR-051

**JR-051** is a biosimilar of agalsidase beta (Fabrazyme), a recombinant human **α-galactosidase A** enzyme replacement therapy (ERT) developed by **JCR Pharmaceuticals** for **Fabry disease**, a rare X-linked lysosomal storage disorder caused by **GLA** gene mutations leading to globotriaosylceramide (GL-3) accumulation in organs like the kidney, heart, and skin. It exhibits bioequivalence to the reference product in pharmacokinetics, pharmacodynamics, and safety, with comparable reductions in plasma GL-3 and lyso-GL-3 levels, identical amino acid sequence, similar glycosylation (higher mannose-6-phosphate content enhancing cellular uptake), and no new safety signals in Phase I, II/III trials and preclinical studies. Administered intravenously, it provides a cost-effective alternative amid reference product shortages.[1][2][4][5][8][10]

Brand names

agalsidase beta BS