Mucopolysaccharidosis type VI: Structura
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Tom Litjens; John J. Hopwood
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Article
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2001
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John Wiley and Sons
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English
β 401 KB
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## Mucopolysaccharidosis type VI (MPS-VI ) is an autosomal recessive lysosomal storage disorder caused by the deficiency of N-acetylgalactosamine-4-sulfatase (4S; or ARSB). Mutations in the 4S gene are responsible for 4S deficiency, which leads to the intralysosomal storage of partially degraded g