Mucopolysaccharidosis type 6 (Q40079): Difference between revisions
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A mucopolissacaridose tipo VI (tipo 6) é uma doença de depósito lisossomal com envolvimento multissistêmico progressivo, associada à deficiência de arilsulfatase B levando ao acúmulo de sulfato de dermatan. O transtorno mostra um amplo espectro de sintomas, desde formas de progressão lenta a rápida. | |||
| description / en | description / en | ||
Mucopolysaccharidosis type 6 (MPS VI) is a lysosomal storage disease with progressive multisystem involvement, associated with a deficiency of arylsulfatase B (ASB) leading to the accumulation of dermatan sulfate. The disorder shows a wide spectrum of symptoms from slowly to rapidly progressing forms. | |||
Revision as of 05:44, 13 August 2026
Mucopolysaccharidosis type 6 (MPS VI) is a lysosomal storage disease with progressive multisystem involvement, associated with a deficiency of arylsulfatase B (ASB) leading to the accumulation of dermatan sulfate. The disorder shows a wide spectrum of symptoms from slowly to rapidly progressing forms.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | 5C56.33 |
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| English | Mucopolysaccharidosis type 6 |
Mucopolysaccharidosis type 6 (MPS VI) is a lysosomal storage disease with progressive multisystem involvement, associated with a deficiency of arylsulfatase B (ASB) leading to the accumulation of dermatan sulfate. The disorder shows a wide spectrum of symptoms from slowly to rapidly progressing forms. |
