Impaired Hematopoiesis and Disrupted Monocyte/Macrophage Homeostasis in Mucopolysaccharidosis Type I Mice.

@article{Viana2016ImpairedHA,
  title={Impaired Hematopoiesis and Disrupted Monocyte/Macrophage Homeostasis in Mucopolysaccharidosis Type I Mice.},
  author={Gustavo Monteiro Viana and Marcus Vin{\'i}cius Buri and Edgar Julian Paredes-Gamero and Ana M Martins and V{\~a}nia D'Almeida},
  journal={Journal of cellular physiology},
  year={2016},
  volume={231 3},
  pages={698-707}
}
Mucopolysaccharidosis type I (MPS I) is a rare autosomal recessive disease caused by alpha-L-iduronidase deficiency in which heparan and dermatan sulfate degradation is compromised. Besides primary lysosomal glycosaminoglycan accumulation, further changes in cellular functions have also been described in several murine MPS models. Herein, we evaluated alterations in hematopoiesis and its implications on the production of mature progeny in a MPS I murine model. Despite the significant increase… CONTINUE READING