Alpha-L-Idosiduronase

Alpha-L-Idosiduronase Uses, Dosage, Side Effects, Food Interaction and all others data.

Human recombinant alpha-L-iduronidase, 628 residues (mature form), produced by recombinant DNAtechnology in a Chinese hamster ovary cell line. Alpha-L-Idosiduronase is a glycoprotein with a molecular weight of approximately 83 kD. The predicted amino acid sequence of the recombinant form, as well as the nucleotide sequence that encodes it, are identical to a polymorphic form of human a-L-iduronidase. It contains 6 N-linked oligosaccharide modification sites.

Alpha-L-Idosiduronase is used to treat mucopolysaccharide storage disorders (specifically mucopolysaccharidosis 1 or Hurlers syndrome) caused by deficiencies of alpha-L-iduronidase. Reduced or absent a-L-iduronidase activity results in the accumulation of the GAG substrates, dermatan sulfate and heparan sulfate, throughout the body and leads to widespread cellular, tissue, and organ dysfunction.

Alpha-L-Idosiduronase
Uses
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Uses during Pregnancy
Uses during Breastfeeding
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Trade Name	Alpha-L-Idosiduronase
Availability	Prescription only
Generic	Laronidase
Laronidase Other Names	alpha-L-Idosiduronase, Human Recombinant alpha-L-iduronidase, Laronidasa, Laronidase, Laronidase (genetical recombination)
Related Drugs	Aldurazyme
Type
Formula	C₃₁₆₀H₄₈₄₈N₈₉₈O₈₈₁S₁₂
Weight	69899.4 Da
Groups	Approved
Therapeutic Class
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Available Country
Last Updated:	September 19, 2023 at 7:00 am

Uses

Alpha-L-Idosiduronase is a form of recombinant human alpha-L-iduronidase used to treat Hurler and Hurler-Scheie forms of mucopolysaccharidosis, a genetic deficiency of alpha-L-iduronidase.

For the treatment of mucopolysaccharidosis

Alpha-L-Idosiduronase is also used to associated treatment for these conditions: Type 1 Mucopolysaccharidosis