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AGE-RELATED FUNCTIONAL AND HISTOPATHOLOGICAL CHANGES OF THE EAR IN THE MPS I MOUSE
OBJECTIVE: Mucopolysaccharidosis type I (MPS I) is an autosomal recessive disorder caused by a mutation in the gene encoding the enzyme α-L-iduronidase. This enzyme is responsible for degradation of dermatan and heparan sulfates. Enzyme deficiency results in their accumulation in lysosomes of virtua...
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| 主要な著者: | , , , , |
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| フォーマット: | Artigo |
| 言語: | Inglês |
| 出版事項: |
2006
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| 主題: | |
| オンライン･アクセス: | https://ncbi.nlm.nih.gov/pmc/articles/PMC1940035/ https://ncbi.nlm.nih.gov/pubmed/17101178 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ijporl.2006.09.016 |
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