Characterization of the MPS I-H knock-in mouse reveals increased femoral biomechanical integrity with compromised material strength and altered bone geometry

Mucopolysaccharidosis type I (MPS I), is an autosomal recessive lysosomal storage disorder caused by a deficiency in the α-L-iduronidase enzyme, resulting in decreased enzymatic activity and accumulation of glycosaminoglycans. The disorder phenotypically manifests with increased urine glycosaminogly...

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Detalles Bibliográficos
Autores principales: Oestreich, Arin K., Garcia, Mekka R., Yao, Xiaomei, Pfeiffer, Ferris M., Nobakhti, Sabah, Shefelbine, Sandra J., Wang, Yong, Brodeur, Amanda C., Phillips, Charlotte L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2015
Materias:
Research Paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5471398/
https://www.ncbi.nlm.nih.gov/pubmed/28649535
http://dx.doi.org/10.1016/j.ymgmr.2015.08.004

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