Наглазим

Наглазим Uses, Dosage, Side Effects, Food Interaction and all others data.

Galsufase is a variant form of the polymorphic human enzyme N-acetylgalactosamine 4-sulfatase of recombinant DNA origin. Наглазим is a glycoprotein with a molecular weight of approximately 56 kD. The recombinant protein is comprised of 495 amino acids and contains six asparagine-linked glycosylation sites, four of which carry a bis mannose-6-phosphate manose7 oligosaccharide for specific cellular recognition. Post-translational modification of Cys53 produces the catalytic amino acid residue Ca-formylglycine, which is required for enzyme activity and is conserved in all members of the sulfatase enzyme family.

Mucopolysaccharide storage disorders are caused by the deficiency of specific lysosomal enzymes required for the catabolism of GAG. Mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome) is characterized by the absence or marked reduction in N-acetylgalactosamine 4-sulfatase. The sulfatase activity deficiency results in the accumulation of the GAG substrate dermatan sulfate, throughout the body. This accumulation leads to widespread cellular, tissue, and organ dysfunction. Наглазим is intended to provide an exogenous enzyme that will be taken up into lysosomes and increase the catabolism of GAG. Наглазим uptake by cells into lysosomes is most likely mediated by the binding of mannose-6-phosphate-terminated oligosaccharide chains of galsulfase to specific mannose-6-phosphate receptors.

Наглазим
Uses
Dosage
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Uses during Pregnancy
Uses during Breastfeeding
Accute Overdose
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Trade Name	Наглазим
Availability	Prescription only
Generic	Galsulfase
Galsulfase Other Names	Arylsulfatase B galsulfase, Galsulfasa, Galsulfase, Galsulfase (genetical recombination)
Related Drugs	Naglazyme
Type
Formula	C₂₅₃₄H₃₈₅₁N₆₉₁O₇₁₉S₁₆
Weight	56012.6 Da
Groups	Approved, Investigational
Therapeutic Class
Manufacturer
Available Country	Russia
Last Updated:	September 19, 2023 at 7:00 am

Uses

Наглазим is a recombinant human enzyme used as replacement enzyme therapy for the treatment of of adults and children with Mucopolysaccharidosis VI, a rare genetic disorder caused by a deficiency of a lysosomal enzyme.

For the treatment of adults and children with Mucopolysaccharidosis VI.

Наглазим is also used to associated treatment for these conditions: Mucopolysaccharidosis VI

How Наглазим works

Наглазим supplies recombinant-engineered galsulfase, a normal variant form of the polymorphic human enzyme, N-acetylgalactosamine 4-sulfatase. It is a lysosomal hydrolase that catalyzes the cleavage of the sulfate ester from terminal N-acetylgalactosamine 4-sulfate residues of GAG chondroitin 4-sulfate and dermatan sulfate. Increased catabolism of GAG in turn reduces systemic dermatan sulfate accumulation, thereby reducing the primary symptoms of MPS VI.