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Evidence of a progressive motor dysfunction in Mucopolysaccharidosis type I mice

Mucopolysaccharidosis (MPS) type I (Hurler syndrome) is a lysosomal storage disorder characterized by deficiency of alpha-L-iduronidase (IDUA), intracellular storage of glycosaminoglycans (GAGs) and progressive neurological pathology. The MPS I mouse model provides an opportunity to study the pathop...

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Detalhes bibliográficos
Main Authors: Baldo, Guilherme, Mayer, Fabiana Quoos, Martinelli, Barbara, Dilda, Anna, Meyer, Fabiola, Ponder, Katherine P., Giugliani, Roberto, Matte, Ursula
Formato: Artigo
Idioma:Inglês
Publicado em: 2012
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3390919/
https://ncbi.nlm.nih.gov/pubmed/22580166
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.bbr.2012.04.051
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