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Regenerative response of skeletal muscles from mice with Marfan Syndrome

Grant number: 14/23391-8
Support Opportunities:Regular Research Grants
Duration: April 01, 2015 - September 30, 2017
Field of knowledge:Biological Sciences - Morphology - Histology
Principal Investigator:Elen Haruka Miyabara
Grantee:Elen Haruka Miyabara
Host Institution: Instituto de Ciências Biomédicas (ICB). Universidade de São Paulo (USP). São Paulo , SP, Brazil


Marfan syndrome (SMF) is an autosomal-dominant systemic disorder of connective tissue, caused by a mutation of fibrillin-1 gene, resulting in changes in the protein fibrillin-1, the major component of microfibrils of the extracellular matrix, present in elastic and non-elastic connective tissues. Although poorly studied, alterations in skeletal muscles from fibrillin-1-deficient mice and from patients with SMF are frequent, such as a decrease in the number and size of myofibers, fibrosis, deposition of adipose tissue, myofiber splitting, hypotonia, decreased strength and susceptibility to fatigue. Although a work has reported that there is a deficit in skeletal muscle regeneration in animals with a mutation of fibrillin-1, the cellular and molecular mechanisms that characterize the impaired muscle regenerative response in these individuals have not been systemically investigated yet. Furthermore, the investigation of possible therapeutic strategies to improve the muscle regenerative potential of these individuals has been poorly explored. The aim of this study is to contribute to a better understanding of the morphological, cellular, molecular and functional aspects of the muscle regenerative process in individuals with SMF, specially the function of satellite cells by using the genetic model that is based on the Cre/loxP system to genetically label satellite cells from animals with SMF (please, verify details in the project); as well as to test possible therapeutic strategies [pharmacological treatment with Ang-(1-7) and transplantation of satellite cells overexpressing the heat shock protein 70 kDa; HSP70] in order to improve the muscle regenerative potential of these individuals. (AU)

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Scientific publications (5)
(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)
SILVA, MEIRICRIS T.; NASCIMENTO, TABATA L.; PEREIRA, MARCELO G.; SIQUEIRA, ADRIANE S.; BRUM, PATRICIA C.; JAEGER, RUY G.; MIYABARA, ELEN H.. beta 2-Adrenoceptor is involved in connective tissue remodeling in regenerating muscles by decreasing the activity of MMP-9. Cell and Tissue Research, v. 365, n. 1, p. 173-186, . (14/13874-1, 14/23391-8, 13/04783-0, 12/21238-2)
DA SILVA, MEIRICRIS TOMAZ; SANTOS, AUDREI R.; KOIKE, TATIANA E.; NASCIMENTO, TABATA L.; ROZANSKI, ANDREI; BOSNAKOVSKI, DARKO; PEREIRA, LYGIA, V; KUMAR, ASHOK; KYBA, MICHAEL; MIYABARA, ELEN H.. The fibrotic niche impairs satellite cell function and muscle regeneration in mouse models of Marfan syndrome. ACTA PHYSIOLOGICA, v. 237, n. 1, p. 19-pg., . (17/14115-5, 20/15351-7, 18/24946-4, 17/09069-4, 14/23391-8, 13/04783-0, 14/13874-1)
KOIKE, TATIANA E.; DELL AQUILA, RODRIGO A.; SILVA, KELLANA S.; AOKI, MARCELO S.; MIYABARA, ELEN H.. Glutamine supplementation improves contractile function of regenerating soleus muscles from rats. JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY, v. 43, n. 2, p. 11-pg., . (17/09069-4, 14/23391-8, 18/24946-4)
KOIKE, TATIANA E.; FUZIWARA, CESAR S.; BRUM, PATRICIA C.; KIMURA, EDNA T.; RANDO, THOMAS A.; MIYABARA, ELEN H.. Muscle Stem Cell Function Is Impaired in beta 2-Adrenoceptor Knockout Mice. STEM CELL REVIEWS AND REPORTS, v. N/A, p. 13-pg., . (17/09069-4, 14/23391-8, 18/24946-4)
NASCIMENTO, TABATA L.; SILVA, MEIRICRIS T.; MIYABARA, ELEN H.. BGP-15 improves contractile function of regenerating soleus muscle. JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY, v. 39, n. 1-2, p. 25-34, . (14/13874-1, 14/23391-8, 13/04783-0)

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