| Grant number: | 10/10458-6 |
| Support Opportunities: | Scholarships in Brazil - Doctorate |
| Start date: | March 01, 2011 |
| End date: | August 31, 2014 |
| Field of knowledge: | Biological Sciences - Biology |
| Principal Investigator: | Vânia D'Almeida |
| Grantee: | Gustavo Monteiro Viana |
| Host Institution: | Escola Paulista de Medicina (EPM). Universidade Federal de São Paulo (UNIFESP). Campus São Paulo. São Paulo , SP, Brazil |
Abstract Mucopolysaccharidosis type I (MPS I) is a hereditary disease caused by a deficiency of alpha-L-iduronidase (IDUA), leading to an impaired glycosaminoglycan (GAG) degradation. When not degraded, GAG accumulates in various tissues and organs, leading to a chronic, progressive and multisystem disease. The cellular mechanisms responsible for the disease etiology, however, are still not fully understood. In a recent paper published by our group (Pereira et al., 2010), alterations in intracellular ion homeostasis and lysosomal permeability were described in Idua -/- mice splenocytes, suggesting the involvement of secondary metabolic and signaling pathways in physiopathology of this disease. The aim of this study is to investigate the relation between ionic homeostasis (intracellular Ca2+ and H+ mobilization), lysosomal permeability (release of cathepsin D into cytosol), autophagy (LC3-I/LC3-II ratio and beclin-1 levels) and apoptosis (caspases activities and pro/anti-apoptotic markers) in primary fibroblasts and hepatocytes cultures from Idua -/- and +/+ mice. The evaluation of alterations on cellular metabolism of this animal model of MPS I bring new perspectives for further studies on patients' cells, as allows a better understanding about the physiopathology of the disease. | |
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