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(Reference retrieved automatically from Web of Science through information on FAPESP grant and its corresponding number as mentioned in the publication by the authors.)

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

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Author(s):
Viana, Gustavo Monteiro [1] ; Buri, Marcus Vinicius [2] ; Paredes-Gamero, Edgar Julian [3, 2] ; Martins, Ana Maria [4] ; D'Almeida, Vania [5, 1]
Total Authors: 5
Affiliation:
[1] Univ Fed Sao Paulo, Dept Pediat, Sao Paulo, SP - Brazil
[2] Univ Fed Sao Paulo, Dept Biochem, Sao Paulo, SP - Brazil
[3] Univ Mogi das Cruzes, Ctr Interdisciplinar Invest Bioquim, Mogi Das Cruzes, SP - Brazil
[4] Univ Fed Sao Paulo, Dept Pediat, Ctr Referencia Erros Inatos Metab, Sao Paulo, SP - Brazil
[5] Univ Fed Sao Paulo, Dept Psychobiol, Sao Paulo - Brazil
Total Affiliations: 5
Document type: Journal article
Source: Journal of Cellular Physiology; v. 231, n. 3, p. 698-707, MAR 2016.
Web of Science Citations: 5
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. (AU)

FAPESP's process: 11/18050-9 - Alterations on cellular homeostasis of mucopolysaccharidosis type I mice
Grantee:Vânia D'Almeida
Support Opportunities: Regular Research Grants
FAPESP's process: 10/10458-6 - Alterations on cellular homeostasis of Mucopolysaccharidosis type I mice fibroblasts and hepatocytes.
Grantee:Gustavo Monteiro Viana
Support Opportunities: Scholarships in Brazil - Doctorate