Delayed hypertrophic differentiation of epiphyseal chondrocytes contributes to failed secondary ossification in mucopolysaccharidosis VII dogs
SH Peck, PJM O'Donnell, JL Kang, NR Malhotra… - Molecular genetics and …, 2015 - Elsevier
Mucopolysaccharidosis (MPS) VII is a lysosomal storage disorder characterized by deficient
β-glucuronidase activity, which leads to the accumulation of incompletely degraded
glycosaminoglycans (GAGs). MPS VII patients present with severe skeletal abnormalities,
which are particularly prevalent in the spine. Incomplete cartilage-to-bone conversion in
MPS VII vertebrae during postnatal development is associated with progressive spinal
deformity and spinal cord compression. The objectives of this study were to determine the …
β-glucuronidase activity, which leads to the accumulation of incompletely degraded
glycosaminoglycans (GAGs). MPS VII patients present with severe skeletal abnormalities,
which are particularly prevalent in the spine. Incomplete cartilage-to-bone conversion in
MPS VII vertebrae during postnatal development is associated with progressive spinal
deformity and spinal cord compression. The objectives of this study were to determine the …
