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

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

Author Viana, Gustavo Monteiro Autor UNIFESP Google Scholar
Buri, Marcus Vinicius Autor UNIFESP Google Scholar
Paredes-Gamero, Edgar Julian Autor UNIFESP Google Scholar
Martins, Ana Maria Autor UNIFESP Google Scholar
D'Almeida, Vania Autor UNIFESP Google Scholar
Abstract 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 in hematopoietic stem cells, a reduction in common myeloid progenitors and granulocyte-macrophage progenitor cells was observed in Idua -/- mice bone marrow. Furthermore, no alterations in number, viability nor activation of cell death mechanisms were observed in Idua -/- mice mature macrophages but they presented higher sensitivity to apoptotic induction after staurosporine treatment. In addition, changes in Ca2+ signaling and a reduction in phagocytosis ability were also found. In summary, our results revealed significant intracellular changes in mature Idua -/- macrophages related to alterations in Idua -/- mice hematopoiesis, revealing a disruption in cell homeostasis. These results provide new insights into physiopathology of MPS I. J. Cell. Physiol. 231: 698-707, 2016. (c) 2015 Wiley Periodicals, Inc.
xmlui.dri2xhtml.METS-1.0.item-coverage Hoboken
Language English
Sponsor Fundacao de Amparo a Pesquisa do Estado de Sao Paulo (FAPESP)
Grant number FAPESP: 2010/10458-6
FAPESP: 2011/18050-9
Date 2016
Published in Journal Of Cellular Physiology. Hoboken, v. 231, n. 3, p. 698-707, 2016.
ISSN 0021-9541 (Sherpa/Romeo, impact factor)
Publisher Wiley
Extent 698-707
Origin http://dx.doi.org/10.1002/jcp.25120
Access rights Closed access
Type Article
Web of Science ID WOS:000365529400020
URI https://repositorio.unifesp.br/handle/11600/57951

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