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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Hauptverfasser: Schachern, Patricia A., Cureoglu, Sebahattin, Tsuprun, Vladimir, Paparella, Michael M., Whitley, Chester
Format: Artigo
Sprache:Inglês
Veröffentlicht: 2006
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Article
Online Zugang: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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