Research Grants 17/15503-9 - Terapia genética, Osteogênese imperfeita - BV FAPESP
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Gene therapy by electrotransfer of mouse growth hormone gene in an animal model of osteogenesis imperfecta

Abstract

Osteogenesis imperfecta (OI), commonly known as brittle bone disease, is a congenital connective tissue dysplasia, mainly characterized by bone deformity, brittleness and low density, short stature, and other connective tissue changes associated with structural or quantitative changes of collagen. In the case of type I OI, there is a decrease in the amount of type I collagen in the extracellular matrix (ECM), since they are heterozygous individuals in which only the non-mutated allele produces this collagen, whereas the mutated allele generates incomplete mRNAs that, due to a nonsense substitution mutation, are degraded before being translated. These individuals have about 50% of the mass of collagen type I compared to normal individuals. The effects of growth hormone (GH) have already been reported in previous studies with an animal model with OI, the oim mice, after administration of recombinant hGH, obtaining a decrease of the fragility and an increase of bone density, with consequent decrease in the number of fractures, increased weight gain, femur and animal lengths. Therefore, considering the experience of our group in this fiels, the main objective of the present work is to evaluate, for the first time, the effects of gene therapy by electrotransfer of the mouse growth hormone (mGH) gene in the oim mice. Studies have shown that GH exerts its anabolic action mainly by increasing the expression of insulin-like growth factor-I (IGF-I), which stimulates the synthesis of procollagens ±1 and ±2. In addition to the parameters mentioned above, catch-up growth and serum mGH and mIGF-I determinations will be also carried out. (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)
LIMA, ELIANA ROSA; CECCHI, CLAUDIA REGINA; HIGUTI, ELIZA; PACHECO DE JESUS, GUSTAVO PROTASIO; GOMES, ALISSANDRA MOURA; ZACARIAS, ENIO APARECIDO; BARTOLINI, PAOLO; PERONI, CIBELE NUNES. Optimization of Mouse Growth Hormone Plasmid DNA Electrotransfer into Tibialis Cranialis Muscle of "Little" Mice. Molecules, v. 25, n. 21, p. 9-pg., . (14/19757-7, 14/07380-6, 17/15503-9)
LIMA, ELIANA ROSA; CECCHI, CLAUDIA REGINA; HIGUTI, ELIZA; PACHECO DE JESUS, GUSTAVO PROTASIO; GOMES, ALISSANDRA MOURA; ZACARIAS, ENIO APARECIDO; BARTOLINI, PAOLO; PERONI, CIBELE NUNES. Optimization of Mouse Growth Hormone Plasmid DNA Electrotransfer into Tibialis Cranialis Muscle of ``Little{''} Mice. Molecules, v. 25, n. 21, . (14/19757-7, 14/07380-6, 17/15503-9)

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