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The role of the neuraminidase-1 on muscle regeneration

Grant number: 11/03853-9
Support type:Scholarships in Brazil - Master
Effective date (Start): August 01, 2011
Effective date (End): July 31, 2013
Field of knowledge:Health Sciences - Medicine
Principal researcher:Edmar Zanoteli
Grantee:Juliana de Carvalho Neves
Home 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)

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Scientific publications
(References retrieved automatically from Web of Science and SciELO through information on FAPESP grants and their corresponding numbers as mentioned in the publications by the authors)
NEVES, JULIANA DE CARVALHO; RIZZATO, VANESSA RODRIGUES; FAPPI, ALAN; GARCIA, MARIANA MIRANDA; CHADI, GERSON; VAN DE VLEKKERT, DIANTHA; D'AZZO, ALESSANDRA; ZANOTELI, EDMAR. Neuraminidase-1 mediates skeletal muscle regeneration. BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE, v. 1852, n. 9, p. 1755-1764, . (09/02937-4, 11/03853-9)
Academic Publications
(References retrieved automatically from State of São Paulo Research Institutions)
NEVES, Juliana de Carvalho. The role of neuraminidase-1 in muscle regeneration. 2014. Master's Dissertation - Universidade de São Paulo (USP). Faculdade de Medicina (FM/SBD) São Paulo.

Please report errors in scientific publications list by writing to: cdi@fapesp.br.