Hurler disease bone marrow stromal cells exhibit altered ability to support osteoclast formation.

@article{Gatto2012HurlerDB,
  title={Hurler disease bone marrow stromal cells exhibit altered ability to support osteoclast formation.},
  author={Francesca Gatto and Daniela Redaelli and Agnese Salvad{\`e} and Simona Marzorati and Benedetto Sacchetti and Chiara Ferina and Valerie Roobrouck and Francesca Bertola and Michela Romano and Guglielmo Villani and Laura Antolini and Attilio Rovelli and Catherine M. Verfaillie and Andrea Biondi and Mara Riminucci and Paolo Bianco and Marta Serafini},
  journal={Stem cells and development},
  year={2012},
  volume={21 9},
  pages={1466-77}
}
Mucopolysaccharidosis type I (MPS IH; Hurler syndrome) is a rare genetic disorder that is caused by mutations in the α-L-iduronidase (IDUA) gene, resulting in the deficiency of IDUA enzyme activity and intra-cellular accumulation of glycosaminoglycans. A characteristic skeletal phenotype is one of the many clinical manifestations in Hurler disease. Since the mechanism(s) underlying these skeletal defects are not completely understood, and bone and cartilage are mesenchymal lineages, we focused… CONTINUE READING

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