Abstract: A method for treating Pompe disease including administration of recombinant human acid ?-glucosidase having optimal glycosylation with mannose-6-phosphate residues in combination with an amount of miglustat effective to maximize tissue uptake of recombinant human acid ?-glucosidase while minimizing inhibition of the enzymatic activity of the recombinant human acid ?-glucosidase is provided.

Abstract: The present application provides for compositions comprising high concentrations of acid ?-glucosidase in combination with an active site-specific chaperone for the acid ?-glucosidase, and methods for treating Pompe disease in a subject in need thereof, that includes a method of administering to the subject such compositions. The present application also provides methods for increasing the in vitro and in vivo stability of an acid ?-glucosidase enzyme formulation.

Abstract: A method for treating Pompe disease including administration of recombinant human acid ?-glucosidase having optimal glycosylation with mannose-6-phosphate residues in combination with an amount of miglustat effective to maximize tissue uptake of recombinant human acid ?-glucosidase while minimizing inhibition of the enzymatic activity of the recombinant human acid ?-glucosidase is provided.

Abstract: The present application provides for compositions comprising high concentrations of acid a-glucosidase in combination with an active site-specific chaperone for the acid ?-glucosidase, and methods for treating Pompe disease in a subject in need thereof, that includes a method of administering to the subject such compositions. The present application also provides methods for increasing the in vitro and in vivo stability of an acid ?-glucosidase enzyme formulation.

Abstract: Provided are a recombinant acid ?-glucosidase and pharmaceutical composition comprising a recombinant acid ?-glucosidase, wherein the recombinant acid ?-glucosidase is expressed in Chinese hamster ovary (CHO) cells and comprises an increased content of N-glycan units bearing one or two mannose-6-phosphate residues when compared to a content of N-glycan units bearing one or two mannose-6-phosphate residues of alglucosidase alfa. Also provided herein are methods of producing, purifying, and formulating the recombinant acid ?-glucosidase or pharmaceutical composition for administration to a subject and methods of treating a disease or disorder such as Pompe disease using the recombinant acid ?-glucosidase or pharmaceutical composition.

Abstract: A method for treating Pompe disease including administration of recombinant human acid ?-glucosidase having optimal glycosylation with mannose-6-phosphate residues in combination with an amount of miglustat effective to maximize tissue uptake of recombinant human acid ?-glucosidase while minimizing inhibition of the enzymatic activity of the recombinant human acid ?-glucosidase is provided.

Abstract: A method for treating Pompe disease including administration of recombinant human acid ?-glucosidase having optimal glycosylation with mannose-6-phosphate residues in combination with an amount of miglustat effective to maximize tissue uptake of recombinant human acid ?-glucosidase while minimizing inhibition of the enzymatic activity of the recombinant human acid ?-glucosidase is provided.

Abstract: The present application provides for compositions comprising high concentrations of acid a-glucosidase in combination with an active site-specific chaperone for the acid ?-glucosidase, and methods for treating Pompe disease in a subject in need thereof, that includes a method of administering to the subject such compositions. The present application also provides methods for increasing the in vitro and in vivo stability of an acid ?-glucosidase enzyme formulation.

Abstract: The present application provides for compositions comprising high concentrations of acid a-glucosidase in combination with an active site-specific chaperone for the acid ?-glucosidase, and methods for treating Pompe disease in a subject in need thereof, that includes a method of administering to the subject such compositions. The present application also provides methods for increasing the in vitro and in vivo stability of an acid ?-glucosidase enzyme formulation.

Abstract: The present application provides for compositions comprising ?-galactosidase A in combination with an active site-specific chaperone for the ?-galactosidase A, and methods for treating Fabry disease in a subject in need thereof, that includes a method of administering to the subject such compositions. The present application also provides methods for increasing the in vitro and in vivo stability of an ?-galactosidase A enzyme formulation.

Abstract: The present application provides for compositions comprising high concentrations of acid a-glucosidase in combination with an active site-specific chaperone for the acid ?-glucosidase, and methods for treating Pompe disease in a subject in need thereof, that includes a method of administering to the subject such compositions. The present application also provides methods for increasing the in vitro and in vivo stability of an acid ?-glucosidase enzyme formulation.

Abstract: The present application provides compositions comprising ?-galactosidase A in combination with an active site-specific chaperone for the ?-galactosidase A, and methods for treating Fabry disease in a subject in need thereof, that includes a method of administering to the subject such compositions. The present application also provides methods for increasing the in vitro and in vivo stability of an ?-galactosidase A enzyme formulation. The present application also provides methods for treating Fabry disease using intravenous administration of 1-deoxygalactonojirimycin.

Abstract: A method for treating Pompe disease including administration of recombinant human acid ?-glucosidase having optimal glycosylation with mannose-6-phosphate residues in combination with an amount of miglustat effective to maximize tissue uptake of recombinant human acid ?-glucosidase while minimizing inhibition of the enzymatic activity of the recombinant human acid ?-glucosidase is provided.

Abstract: The present application provides for compositions comprising high concentrations of acid a-glucosidase in combination with an active site-specific chaperone for the acid ?-glucosidase, and methods for treating Pompe disease in a subject in need thereof, that includes a method of administering to the subject such compositions. The present application also provides methods for increasing the in vitro and in vivo stability of an acid ?-glucosidase enzyme formulation.

Abstract: The present application provides for compositions comprising ?-galactosidase A in combination with an active site-specific chaperone for the ?-galactosidase A, and methods for treating Fabry disease in a subject in need thereof, that includes a method of administering to the subject such compositions. The present application also provides methods for increasing the in vitro and in vivo stability of an ?-galactosidase A enzyme formulation.

Abstract: The present application provides compositions comprising ?-galactosidase A in combination with an active site-specific chaperone for the ?-galactosidase A, and methods for treating Fabry disease in a subject in need thereof, that includes a method of administering to the subject such compositions. The present application also provides methods for increasing the in vitro and in vivo stability of an ?-galactosidase A enzyme formulation. The present application also provides methods for treating Fabry disease using intravenous administration of 1-deoxygalactonojirimycin.

Abstract: A method for treating Pompe disease including administration of recombinant human acid ?-glucosidase having optimal glycosylation with mannose-6-phosphate residues in combination with an amount of miglustat effective to maximize tissue uptake of recombinant human acid ?-glucosidase while minimizing inhibition of the enzymatic activity of the recombinant human acid ?-glucosidase is provided.

Abstract: The present application provides for compositions comprising ?-galactosidase A in combination with an active site-specific chaperone for the ?-galactosidase A, and methods for treating Fabry disease in a subject in need thereof, that includes a method of administering to the subject such compositions. The present application also provides methods for increasing the in vitro and in vivo stability of an ?-galactosidase A enzyme formulation.

Abstract: The present application provides for compositions comprising high concentrations of acid ?-glucosidase in combination with an active site-specific chaperone for the acid ?-glucosidase, and methods for treating Pompe disease in a subject in need thereof, that includes a method of administering to the subject such compositions. The present application also provides methods for increasing the in vitro and in vivo stability of an acid ?-glucosidase enzyme formulation.

Abstract: The present application provides for compositions comprising high concentrations of acid ?-glucosidase in combination with an active site-specific chaperone for the acid ?-glucosidase, and methods for treating Pompe disease in a subject in need thereof, that includes a method of administering to the subject such compositions. The present application also provides methods for increasing the in vitro and in vivo stability of an acid ?-glucosidase enzyme formulation.