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Published in 2018 at "Biotechnology and Applied Biochemistry"
DOI: 10.1002/bab.1660
Abstract: Hunter syndrome (Mucopolysaccharidosis II, MPS II) is an X‐linked lysosomal storage disease produced by the deficiency of the lysosomal enzyme iduronate‐2‐sulfatase (IDS). Currently, MPS II patients are mainly treated with enzyme replacement therapy (ERT) using…
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Keywords:
production;
enzyme;
production characterization;
pichia pastoris ... See more keywords
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Published in 2019 at "Heliyon"
DOI: 10.1016/j.heliyon.2019.e01667
Abstract: Iduronate-2-sulfatase (IDS) is a lysosomal enzyme involved in the metabolism of the glycosaminoglycans heparan (HS) and dermatan (DS) sulfate. Mutations on IDS gene produce mucopolysaccharidosis II (MPS II), characterized by the lysosomal accumulation of HS…
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Keywords:
mouse brain;
type mouse;
brain;
wild type ... See more keywords
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Published in 2020 at "DNA and cell biology"
DOI: 10.1089/dna.2019.5221
Abstract: Hunter's syndrome (mucopolysaccharidosis type II) is a rare X-linked lysosomal storage disorder caused by mutations in the iduronate-2-sulfatase (IDS) gene. Motivated by the case of a child affected by this syndrome, we compared the intracellular…
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Keywords:
hunter syndrome;
lysosomal quality;
control;
quality control ... See more keywords
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Published in 2023 at "Human gene therapy"
DOI: 10.1089/hum.2023.025
Abstract: Mucopolysaccharidosis type II (MPSII) is a lysosomal storage disease caused by a mutation in the IDS gene, resulting in deficiency of the enzyme iduronate-2-sulfatase (IDS) causing heparan sulfate (HS) and dermatan sulfate (DS) accumulation in…
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Keywords:
iduronate sulfatase;
rvg gh625;
mucopolysaccharidosis type;
type ... See more keywords