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Decreased performance in IDUA knockout mouse mimic limitations of joint function and locomotion in patients with Hurler syndrome

BACKGROUND: Mucopolysaccharidosis type I (MPS I) is caused by the deficiency of alpha-L-iduronidase (IDUA), which is involved in the degradation of glycosaminoglycans (GAGs), such as heparan sulfate and dermatan sulfate in the lysosome. It has been reported that joint symptoms are almost universal i...

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Detalles Bibliográficos
Autores principales: Kim, Chihwa, Kwak, Min Jung, Cho, Sung Yoon, Ko, Ah-ra, Rheey, Jinguen, Kwon, Jeong-Yi, Chung, Yokyung, Jin, Dong-Kyu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4582722/
https://www.ncbi.nlm.nih.gov/pubmed/26407983
http://dx.doi.org/10.1186/s13023-015-0337-3