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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...
Autores principales: | Kim, Chihwa, Kwak, Min Jung, Cho, Sung Yoon, Ko, Ah-ra, Rheey, Jinguen, Kwon, Jeong-Yi, Chung, Yokyung, Jin, Dong-Kyu |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2015
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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 |
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