Hydrolytic Lysosomal Glycosaminoglycan-specific Enzyme

Mucopolysaccharidosis VI

<section class="Section" data-sectioncode="34089-3"><a name="LINK_8b8102cf-1fa5-41a5-9321-80889df7d475"></a><a name="section-8"></a><p></p><h1>11 DESCRIPTION</h1><p class="First">NAGLAZYME is a formulation of galsulfase, which is a purified human enzyme that is produced by recombinant DNA technology in a Chinese hamster ovary cell line. <span class="Italics"> </span>Galsulfase (glycosaminoglycan <span class="Italics">N</span>-acetylgalactosamine 4-sulfatase, EC 3.1.6.12) is a lysosomal enzyme that catalyzes the cleavage of the sulfate ester from terminal <span class="Italics">N</span>–acetylgalactosamine 4-sulfate residues of glycosaminoglycans (GAG), chondroitin 4-sulfate and dermatan sulfate.</p><p>Galsulfase is a glycoprotein with a molecular weight of approximately 56 kDa.  The recombinant protein consists of 495 amino acids and possesses six asparagine‑linked glycosylation sites, four of which carry a bis‑mannose-6–phosphate residue for specific cellular recognition.  Post-translational modification of Cys53 produces the catalytic amino acid residue, Cα-formylglycine, which is required for enzyme activity.  NAGLAZYME has a specific activity of approximately 70 units per mg of protein content.  One activity unit is defined as the amount of enzyme required to convert 1 micromole of 4-methylumbelliferyl sulfate to 4-methylumbelliferone and free sulfate per minute at 37°C.</p><p>NAGLAZYME is intended for intravenous infusion and is supplied as a sterile, nonpyrogenic, colorless to pale yellow, clear to slightly opalescent solution that must be diluted with 0.9% Sodium Chloride Injection, USP, prior to administration.  NAGLAZYME is supplied in clear Type I glass 5 mL vials.  Each vial provides 5 mg galsulfase, 43.8 mg sodium chloride, 6.20 mg sodium phosphate monobasic monohydrate, 1.34 mg sodium phosphate dibasic heptahydrate, and 0.25 mg polysorbate 80 in a 5 mL extractable solution with pH of approximately 5.8.  NAGLAZYME does not contain preservatives. Each vial is for single use only.</p></section>

NAGLAZYME

galsulfase

5 ML galsulfase 1 MG/ML Injection

Naglazyme

5 ML galsulfase 1 MG/ML Injection [Naglazyme]

Psoriasis (PsO)

Psoriatic Arthritis (PsA)

Ankylosing Spondylitis (AS)

Nonradiographic Axial Spondyloarthritis

Taltz

Juvenile Idiopathic Arthritis

Hidradenitis Suppurativa (HS)

Cosentyx

Siliq

Tremfya

Crohn's Disease (CD)

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Coverage Authorization Guide

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Letter of Medical Necessity

BioMarin RareConnections Enrollment Form 

BioMarin RareConnections Patient Consent Form - To be used along with BioMarin RareConnections Form 