-
Iduronidase (EC 3.2.1.76, L-
iduronidase, α-L-
iduronidase, laronidase), sold as Aldurazyme, is an
enzyme with the
systematic name
glycosaminoglycan α-L-iduronohydrolase...
- eyes, and heart. The
underlying mechanism is a
deficiency of alpha-L
iduronidase, an
enzyme responsible for
breaking down GAGs.: 544
Without this enzyme...
-
glycosaminoglycans (or GAGs, or mucopolysaccharides) due to a
deficiency of alpha-L
iduronidase, an
enzyme responsible for the
degradation of GAGs in lysosomes. Without...
-
enzyme alpha-L-
iduronidase.
Children born to an MPS I
parent carry the
defective gene. MPS I H (also
called Hurler syndrome or α-L-
iduronidase deficiency)...
-
Scheie syndrome is a
disease caused by a
deficiency in the
enzyme iduronidase,
leading to the
buildup of
glycosaminoglycans (GAGs) in the body. It is...
- to
break down GAGs in
their lysosomes due a
deficiency of the
enzyme iduronidase.[citation needed] All
forms of
mucopolysaccharidosis type I (MPS I) are...
- (MPS I H, α-L-
iduronidase deficiency)
Scheie syndrome (MPS I S) Hurler–Scheie
syndrome (MPS I H-S)
Hunter syndrome (MPS II,
iduronidase sulfate deficiency)...
- D., Neufeld, E.F.:
Carbohydrate structures of
recombinant human a-L-
iduronidase secreted by
Chinese hamster ovary cells. The
Journal of
Biological Chemistry...
-
collagen disease Alpha-ketoglutarate
dehydrogenase deficiency Alpha-L-
iduronidase deficiency Alpha-mannosidosis Alpha-sarcoglycanopathy Alpha-thal****emia...
- Maroteaux–Lamy
syndrome (also
called mucopolysaccharidosis type VI)
Iduronidase (branded as Aldurazyme), a
recombinant protein therapeutic for mucopolysaccharidosis...