Recombinant alpha-L-iduronidase: characterization of the purified enzyme and correction of mucopolysaccharidosis type I fibroblasts.

Mucopolysaccharidosis type I (MPS I, Hurler and Scheie syndromes) is an autosomal recessive lysosomal storage disorder that results from a deficiency of the hydrolase alpha-L-iduronidase (IDUA) which is involved in the lysosomal degradation of both heparan sulphate (HS) and dermatan sulphate (DS). P...

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Bibliographic Details
Main Authors: Unger, E G, Durrant, J, Anson, D S, Hopwood, J J
Format: Artigo
Language:Inglês
Published: 1994
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Research Article
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC1137449/
https://ncbi.nlm.nih.gov/pubmed/7998955
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