Please use this identifier to cite or link to this item: https://hdl.handle.net/2440/7090
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Type: Journal article
Title: Histomorphometric analysis of the tibial growth plate in a feline model of mucopolysaccharidosis type VI
Author: Nuttall, J.
Brumfield, L.
Fazzalari, N.
Hopwood, J.
Byers, S.
Citation: Calcified Tissue International, 1999; 65(1):47-52
Publisher: SPRINGER VERLAG
Issue Date: 1999
ISSN: 0171-967X
1432-0827
Abstract: Mucopolysaccharidosis type VI (MPS VI) is a genetically inherited lysosomal storage disorder. Severely affected children exhibit a range of skeletal abnormalities including short stature, facial dysmorphia, and dysostosis multiplex. Naturally occurring and transgenic animal models of MPS VI are also found which exhibit pathology similar to the human disorder. In this paper we have characterized the formation of trabecular bone from growth plate cartilage in a feline model of MPS VI. Tibial trabecular bone was shown to be osteopenic in MPS VI animals with a bone mineral volume (BV/TV) of 4.51% compared with a BV/TV of 15.64% in normal animals. In addition to osteopenia, a rearrangement of trabecular bone architecture was also observed in MPS VI tibiae, with fewer, thinner trabeculae noted; bone formation rate was also decreased. These observations support those previously made in the L5 vertebrae of MPS VI animals. When the sequential formation of growth plate cartilage structural elements, their transition into primary bone spongiosa, and remodeling into secondary bone spongiosa was characterized, no difference between normal and MPS VI could be detected in the number of cartilage septae and their arrangement in the proliferative and hypertrophic regions of the growth plate or trabecular elements in the primary spongiosa. However, a deviation from normal was observed in the resting zone of the growth plate and in the secondary spongiosa of bone. Thus, the osteopenia observed in MPS VI bone appears to arise primarily from a defect in bone production within the metaphysis and diaphysis rather than the creation of an abnormal template in the preceding growth plate cartilage.
Keywords: Growth Plate
Tibia
Animals
Cats
Bone Diseases, Metabolic
Mucopolysaccharidosis VI
Disease Models, Animal
Osteogenesis
Bone Density
DOI: 10.1007/s002239900656
Appears in Collections:Aurora harvest 5
Paediatrics publications

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