Articles with "iduronate sulfatase" as a keyword



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Production and characterization of a human lysosomal recombinant iduronate‐2‐sulfatase produced in Pichia pastoris

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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… read more here.

Keywords: production; enzyme; production characterization; pichia pastoris ... See more keywords
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Identification of the iduronate-2-sulfatase proteome in wild-type mouse brain

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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… read more here.

Keywords: mouse brain; type mouse; brain; wild type ... See more keywords
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Endoplasmic Reticulum and Lysosomal Quality Control of Four Nonsense Mutants of Iduronate 2-Sulfatase Linked to Hunter's Syndrome.

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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… read more here.

Keywords: hunter syndrome; lysosomal quality; control; quality control ... See more keywords
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Fusion of RVG or gh625 to Iduronate-2-Sulfatase for the Treatment of Mucopolysaccharidosis Type II.

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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… read more here.

Keywords: iduronate sulfatase; rvg gh625; mucopolysaccharidosis type; type ... See more keywords