Upregulation of elastase activity in aorta in mucopolysaccharidosis I and VII dogs may be due to increased cytokine expression.

@article{Metcalf2010UpregulationOE,
  title={Upregulation of elastase activity in aorta in mucopolysaccharidosis I and VII dogs may be due to increased cytokine expression.},
  author={Jason A Metcalf and Bruce Linders and Susan Y. J. Wu and Paul W Bigg and Patricia O'donnell and Meg M. Sleeper and Michael P Whyte and Mark E. Haskins and Katherine Parker Ponder},
  journal={Molecular genetics and metabolism},
  year={2010},
  volume={99 4},
  pages={396-407}
}
Mucopolysaccharidosis I (MPS I) and MPS VII are due to loss-of-function mutations within the genes that encode the lysosomal enzymes alpha-l-iduronidase and beta-glucuronidase, respectively, and result in accumulation of glycosaminoglycans and multisystemic disease. Both disorders are associated with elastin fragmentation and dilatation of the aorta. Here, the pathogenesis and effect of gene therapy on aortic disease in canine models of MPS was evaluated. We found that cathepsin S is… CONTINUE READING
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