Altered lumbar spine structure, biochemistry, and biomechanical properties in a canine model of mucopolysaccharidosis type VII.

Journal of Orthopaedic Research : Official Publication of the Orthopaedic Research Society
Lachlan J SmithDawn M Elliott

Abstract

Mucopolysaccharidosis VII (MPS VII) is a lysosomal storage disorder characterized by a deficiency in beta-glucuronidase activity, leading to systemic accumulation of poorly degraded glycosaminoglycans (GAG). Along with other morbidities, MPS VII is associated with pediatric spinal deformity. The objective of this study was to examine potential associations between abnormal lumbar spine matrix structure and composition in MPS VII, and spine segment and tissue-level mechanical properties, using a naturally occurring canine model with a similar clinical phenotype to the human form of the disorder. Segments from juvenile MPS VII and unaffected dogs were allocated to: radiography, gross morphology, histology, biochemistry, and mechanical testing. MPS VII spines had radiolucent lesions in the vertebral body epiphyses. Histologically, this corresponded to a GAG-rich cartilaginous region in place of bone and elevated GAG staining was seen in the annulus fibrosus. Biochemically, MPS VII samples had elevated GAG in the outer annulus fibrosus and epiphyses, low calcium in the epiphyses, and high water content in all regions except the nucleus pulposus. MPS VII spine segments had higher range of motion and lower stiffness than controls. En...Continue Reading

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Citations

Apr 25, 2012·Molecular Genetics and Metabolism·Carmen E MacsaiSharon Byers
Jul 21, 2011·Journal of Tissue Engineering and Regenerative Medicine·Mihael LazebnikMichael S Detamore
Nov 30, 2012·Journal of Orthopaedic Research : Official Publication of the Orthopaedic Research Society·Alon LaiJames C Iatridis
Jun 23, 2021·Molecular Genetics and Metabolism·Sun H PeckLachlan J Smith
Oct 17, 2020·Current Osteoporosis Reports·Zhirui JiangLachlan J Smith
Apr 20, 2012·Molecular Genetics and Metabolism·Lachlan J SmithKatherine P Ponder

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