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

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dc.contributor.author Viana, Gustavo Monteiro [UNIFESP]
dc.contributor.author Buri, Marcus Vinicius [UNIFESP]
dc.contributor.author Paredes-Gamero, Edgar Julian [UNIFESP]
dc.contributor.author Martins, Ana Maria [UNIFESP]
dc.contributor.author D'Almeida, Vania [UNIFESP]
dc.date.accessioned 2020-08-21T17:00:21Z
dc.date.available 2020-08-21T17:00:21Z
dc.date.issued 2016
dc.identifier http://dx.doi.org/10.1002/jcp.25120
dc.identifier.citation Journal Of Cellular Physiology. Hoboken, v. 231, n. 3, p. 698-707, 2016.
dc.identifier.issn 0021-9541
dc.identifier.uri https://repositorio.unifesp.br/handle/11600/57951
dc.description.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. en
dc.description.sponsorship Fundacao de Amparo a Pesquisa do Estado de Sao Paulo (FAPESP)
dc.format.extent 698-707
dc.language.iso eng
dc.publisher Wiley
dc.relation.ispartof Journal Of Cellular Physiology
dc.rights Acesso restrito
dc.title Impaired Hematopoiesis and Disrupted Monocyte/Macrophage Homeostasis in Mucopolysaccharidosis Type I Mice en
dc.type Artigo
dc.description.affiliation Univ Fed Sao Paulo, Dept Pediat, Sao Paulo, SP, Brazil
dc.description.affiliation Univ Fed Sao Paulo, Dept Biochem, Sao Paulo, SP, Brazil
dc.description.affiliation Univ Mogi das Cruzes, Ctr Interdisciplinar Invest Bioquim, Mogi Das Cruzes, SP, Brazil
dc.description.affiliation Univ Fed Sao Paulo, Dept Pediat, Ctr Referencia Erros Inatos Metab, Sao Paulo, SP, Brazil
dc.description.affiliation Univ Fed Sao Paulo, Dept Psychobiol, Sao Paulo, Brazil
dc.description.affiliationUnifesp Univ Fed Sao Paulo, Dept Pediat, Sao Paulo, SP, Brazil
dc.description.affiliationUnifesp Univ Fed Sao Paulo, Dept Biochem, Sao Paulo, SP, Brazil
dc.description.affiliationUnifesp Univ Fed Sao Paulo, Dept Pediat, Ctr Referencia Erros Inatos Metab, Sao Paulo, SP, Brazil
dc.description.affiliationUnifesp Univ Fed Sao Paulo, Dept Psychobiol, Sao Paulo, Brazil
dc.description.sponsorshipID FAPESP: 2010/10458-6
dc.description.sponsorshipID FAPESP: 2011/18050-9
dc.identifier.doi 10.1002/jcp.25120
dc.description.source Web of Science
dc.identifier.wos WOS:000365529400020
dc.coverage Hoboken
dc.citation.volume 231
dc.citation.issue 3



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