| Grant number: | 11/03853-9 |
| Support Opportunities: | Scholarships in Brazil - Master |
| Start date: | August 01, 2011 |
| End date: | July 31, 2013 |
| Field of knowledge: | Health Sciences - Medicine |
| Principal Investigator: | Edmar Zanoteli |
| Grantee: | Juliana de Carvalho Neves |
| Host Institution: | Faculdade de Medicina (FM). Universidade de São Paulo (USP). São Paulo , SP, Brazil |
Abstract Neuraminidase-1 (Neu1) regulates the catabolism of sialoglycoconjugates in lysosomes. Congenital Neu1 deficiency in children is the basis of sialidosis, a neurosomatic disorder whose symptoms include hypotonia, muscle weakness and osteoskeletal deformities. Mice with Neu1 deficiency develop an atypical form of muscle degeneration characterized by abnormal fibroblast proliferation and expanded extra cellular matrix (ECM), with invasion of muscle fibers by ECM components, cytosolic fragmentation, vacuolar formation and muscle atrophy. The aim of this study is to investigate the role of Neu1 on muscle regeneration process. Experimentally, the satellite cells proliferation, inflammatory response, muscle fiber maturation and fibrosis development during the muscle regeneration process induced by intramuscular administration of cardiotoxin will be assessed in Neu1 deficient mice. The determination of Neu1 role on muscle physiology will be the great value to understand the neuromuscular clinical manifestations reported in patients with Neu1 deficiency and the importance of lysosomes and the sialic acid metabolism on the physiopathogeneses of muscle diseases. (AU) | |
| News published in Agência FAPESP Newsletter about the scholarship: | |
| More itemsLess items | |
| TITULO | |
| Articles published in other media outlets ( ): | |
| More itemsLess items | |
| VEICULO: TITULO (DATA) | |
| VEICULO: TITULO (DATA) | |