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

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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Bibliographic Details
Main Authors: Arin K. Oestreich, Mekka R. Garcia, Xiaomei Yao, Ferris M. Pfeiffer, Sabah Nobakhti, Sandra J. Shefelbine, Yong Wang, Amanda C. Brodeur, Charlotte L. Phillips
Format: Article
Language:English
Published: Elsevier 2015-12-01
Series:Molecular Genetics and Metabolism Reports
Subjects:
Bone biomechanics
Raman spectroscopy
Mucopolysaccharidosis type I
Idua-W392X
α-L-iduronidase
Online Access:http://www.sciencedirect.com/science/article/pii/S2214426915300288

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