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Oestreich AK, Garcia MR, Yao X, et al. Characterization of the MPS I-H knock-in mouse reveals increased femoral biomechanical integrity with compromised material strength and altered bone geometry. Mol Genet Metab Rep. 2015;5:3-11doi: 10.1016/j.ymgmr.2015.08.004.
Oestreich, A. K., Garcia, M. R., Yao, X., Pfeiffer, F. M., Nobakhti, S., Shefelbine, S. J., Wang, Y., Brodeur, A. C., & Phillips, C. L. (2015). Characterization of the MPS I-H knock-in mouse reveals increased femoral biomechanical integrity with compromised material strength and altered bone geometry. Molecular genetics and metabolism reports, 53-11. https://doi.org/10.1016/j.ymgmr.2015.08.004
Oestreich, Arin K, et al. "Characterization of the MPS I-H knock-in mouse reveals increased femoral biomechanical integrity with compromised material strength and altered bone geometry." Molecular genetics and metabolism reports vol. 5 (2015): 3-11. doi: https://doi.org/10.1016/j.ymgmr.2015.08.004
Oestreich AK, Garcia MR, Yao X, Pfeiffer FM, Nobakhti S, Shefelbine SJ, Wang Y, Brodeur AC, Phillips CL. Characterization of the MPS I-H knock-in mouse reveals increased femoral biomechanical integrity with compromised material strength and altered bone geometry. Mol Genet Metab Rep. 2015 Sep 07;5:3-11. doi: 10.1016/j.ymgmr.2015.08.004. eCollection 2015 Dec. PMID: 28649535; PMCID: PMC5471398.
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Mol Genet Metab Rep. 2015 Sep 07;5:3-11. doi: 10.1016/j.ymgmr.2015.08.004. eCollection 2015 Dec.

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

Molecular genetics and metabolism reports

Arin K Oestreich, Mekka R Garcia, Xiaomei Yao, Ferris M Pfeiffer, Sabah Nobakhti, Sandra J Shefelbine, Yong Wang, Amanda C Brodeur, Charlotte L Phillips

Affiliations

  1. Department of Biological Sciences, University of Missouri, Columbia, MO 65211, United States.
  2. Department of Biochemistry, University of Missouri, Columbia, MO 65211, United States.
  3. Department of Oral and Craniofacial Sciences, School of Dentistry, University of Missouri-Kansas City, Kansas City, MO 64108, United States.
  4. Department of Orthopaedic Surgery and Bioengineering, University of Missouri, Columbia, MO 65211, United States.
  5. Department of Mechanical and Industrial Engineering, Northeastern University, Boston, MA 02115, United States.
  6. Department of Biomedical Sciences, Missouri State University, Springfield, MO 65804, United States.
  7. Department of Child Health, University of Missouri, Columbia, MO 65211, United States.

PMID: 28649535 PMCID: PMC5471398 DOI: 10.1016/j.ymgmr.2015.08.004

Abstract

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 glycosaminoglycan excretion, facial dysmorphology, neuropathology, cardiac manifestations, and bone deformities. While the development of new treatment strategies have shown promise in attenuating many symptoms associated with the disorder, the bone phenotype remains unresponsive. The aim of this study was to investigate and further characterize the skeletal manifestations of the

Keywords: BMD, bone mineral density; BMDD, bone mineral density distribution; BV/TV, bone volume/total volume; Bone biomechanics; FWHM, full width at half maximum; G, shear modulus of elasticity; GAGs, glycosaminoglycans; IDUA, α-L-iduronidase; Idua-W392X; Ks, stiffness; MPS I, mucopolysaccharidosis type I; Mucopolysaccharidosis type I; Raman spectroscopy; SMI, structure model index; Su, tensile strength; Tmax, torsional ultimate strength; U, energy to failure; α-L-iduronidase; μCT, microcomputed tomography

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