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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\u2010macrophage progenitor cells was observed in <jats:italic>Idua<\/jats:italic> \u2212\/\u2212 mice bone marrow. Furthermore, no alterations in number, viability nor activation of cell death mechanisms were observed in <jats:italic>Idua<\/jats:italic> \u2212\/\u2212 mice mature macrophages but they presented higher sensitivity to apoptotic induction after staurosporine treatment. In addition, changes in Ca<jats:sup>2+<\/jats:sup> signaling and a reduction in phagocytosis ability were also found. In summary, our results revealed significant intracellular changes in mature <jats:italic>Idua<\/jats:italic> \u2212\/\u2212 macrophages related to alterations in <jats:italic>Idua<\/jats:italic> \u2212\/\u2212 mice hematopoiesis, revealing a disruption in cell homeostasis. These results provide new insights into physiopathology of MPS I. J. Cell. Physiol. 231: 698\u2013707, 2016. \u00a9 2015 Wiley Periodicals, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jcp.25120","type":"journal-article","created":{"date-parts":[[2015,8,3]],"date-time":"2015-08-03T12:17:53Z","timestamp":1438604273000},"page":"698-707","source":"Crossref","is-referenced-by-count":11,"title":["Impaired Hematopoiesis and Disrupted Monocyte\/Macrophage Homeostasis in Mucopolysaccharidosis Type I Mice"],"prefix":"10.1002","volume":"231","author":[{"given":"Gustavo Monteiro","family":"Viana","sequence":"first","affiliation":[{"name":"Department of Pediatrics Universidade Federal de S\u00e3o Paulo S\u00e3o Paulo S\u00e3o Paulo Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marcus Vin\u00edcius","family":"Buri","sequence":"additional","affiliation":[{"name":"Department of Biochemistry Universidade Federal de S\u00e3o Paulo S\u00e3o Paulo S\u00e3o Paulo Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Edgar Julian","family":"Paredes\u2010Gamero","sequence":"additional","affiliation":[{"name":"Department of Biochemistry Universidade Federal de S\u00e3o Paulo S\u00e3o Paulo S\u00e3o Paulo Brazil"},{"name":"Centro Interdisciplinar de Investiga\u00e7\u00e3o Bioqu\u00edmica Universidade de Mogi das Cruzes Mogi das Cruzes S\u00e3o Paulo Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana Maria","family":"Martins","sequence":"additional","affiliation":[{"name":"Centro de Refer\u00eancia de Erros Inatos do Metabolismo (CREIM) Department of Pediatrics Universidade Federal de S\u00e3o Paulo S\u00e3o Paulo S\u00e3o Paulo Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"V\u00e2nia","family":"D'Almeida","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Universidade Federal de S\u00e3o Paulo S\u00e3o Paulo S\u00e3o Paulo Brazil"},{"name":"Department of Psychobiology Universidade Federal de S\u00e3o Paulo S\u00e3o Paulo S\u00e3o Paulo Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2015,11,23]]},"reference":[{"key":"e_1_2_6_2_1","first-page":"S34","article-title":"Disease pathogenesis explained by basic science: Lysosomal storage diseases as autophagocytic disorders","volume":"47","author":"Ballabio A","year":"2009","journal-title":"Int J Clin Pharmacol Ther"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbamcr.2008.12.001"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-005-0136-4"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004390100606"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M200877200"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1705718"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11011-005-4152-8"},{"key":"e_1_2_6_9_1","first-page":"18","article-title":"The mucopolysaccharidosis: A success of molecular medicine","author":"Clarke LA","year":"2008","journal-title":"Expert Rev Mol Med"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/6.4.503"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/ni.2705"},{"key":"e_1_2_6_12_1","first-page":"3488","article-title":"Analysis of male reproductive parameters in a murine model of mucopolysaccharidosis type I (MPS I)","volume":"7","author":"do Nascimento CC","year":"2014","journal-title":"Int J Clin Exp Pathol"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/emboj.2012.170"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrm1423"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1703395"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.2006.124719"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuint.2013.03.007"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.4161\/auto.3906"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ceca.2007.12.005"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1152\/physiol.00025.2005"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1158\/2326-6066.CIR-13-0117"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)52451-6"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1042\/BST0351088"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2008-0416"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.prrv.2010.10.005"},{"key":"e_1_2_6_26_1","unstructured":"NeufeldEF MuenzerJ.2001. 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