Long-Term Preservation of Cones and Improvement in Visual Function Following Gene Therapy in a Mouse Model of Leber Congenital Amaurosis Caused by Guanylate Cyclase-1 Deficiency

Leber congenital amaurosis (LCA) is a severe retinal dystrophy manifesting from early infancy as poor vision or blindness. Loss-of-function mutations in GUCY2D cause LCA1 and are one of the most common causes of LCA, accounting for 20% of all cases. Human GUCY2D and mouse Gucy2e genes encode guanylate cyclase-1 (GC), which is responsible for restoring the dark state in photoreceptors after light exposure. The Glicy2e(-/-) mouse shows partially diminished rod function, but an absence of cone function before degeneration. Although the cones appear morphologically normal, they exhibit mislocalization of proteins involved in phototransduction. In this study we tested the efficacy of an rAAV2/8 vector containing the human rhodopsin kinase promoter and the human GUCY2D gene. Following subretinal delivery of the vector in Glicy2e(-/-) mice, GC1 protein was detected in the rod and cone outer segments, and in transduced areas of retina cone transducin was appropriately localized to cone outer segments. Moreover, we observed a dose-dependent restoration of rod and cone function and an improvement in visual behavior of the treated mice. Most importantly, cone preservation was observed in transduced areas up to 6 months post injection. To date, this is the most effective rescue of the Glicy2e(-/-) mouse model of LCA and we propose that a vector, similar to the one used in this study, could be suitable for use in a clinical trial of gene therapy for LCA1

Law Enforcement At Issue: Constitutionality Of The Maria Da Penha Law In Brazilian Courts

The objective of this study was to identify the main positions regarding the constitutionality of the Maria da Penha law (Law 11340/2006) in the Brazilian judicial system. As a result of political demands of the Brazilian feminist movement, the law has been at issue in the public sphere, and its constitutionality before the Supreme Federal Court has been pursued. Th e following issues were identifi ed: i) a questioning of the law as a whole, considering its distinguished treatment of women, ii) a questioning of the law in that it precludes the enforcement of Law 9099/95; iii) the legislative competence to defi ne petty crimes, iv) the support for subjection of the Judicial Branch and v) the constitutionality of law without background reasoning. By examining the arguments used in Courts of Justice, we intend to demonstrate how the establishment of the law is not limited to the legislative act, and the Judiciary can be the stage for disputes.9166183Dias, M.B., Um ano sem festa (2007) Folha de S.Paulo, pp. A3. , São Paulo, 22 set. 2007. Caderno principal Tendências e DebatesFreire, N., Lei Maria da Penha, já (2008) Folha de S.Paulo, , São Paulo, 7 ago. 2008. Caderno principal, Tendências e Debates, p. A3Genro, T., Erradicar a violência contra a mulher, já (2008) Folha de S.Paulo, pp. A3. , São Paulo, 9 mar. 2008. Caderno principal, Tendências e DebatesMiraglia, P., Incapaz de prevenir, incapaz de proteger (2011) Último Segundo Online, , ultimosegundo.ig.com.br/colunistas/paulamiraglia/incapaz+de+prevenir+incapaz+de+proteger/c1300098763543.html, 24 abr. 2011. Available at: Last accessed: 18 May. 2011(2010) O Estado de S.Paulo Online, , www.estadao.com.br/noticias/nacional,para-especialistas-brasil-falha-na-aplicacao-de-leis-de-protecao-amulher,579003,0.htm, PARA especialistas, Brasil falha na aplicação de leis de proteção à mulher. São Paulo, 9 jul. 2010. Available at: Last accessed: 24 Out. 2011Rodriguez, J.R., The Persistence of Formalism: Towards a Situated Critique beyond the Classic Separation of Powers (2010) Law and Development Review, 3 (2)Rodriguez, J.R., Zonas de autarquia nas decisões jurisdicionais: Estado de Direito, indeterminação e democracia (2011) O Estado de Direito e os Desafios do Desenvolvimento, , VILHENA, O.V.DIMOULIS, D. São Paulo: SaraivaRodriguez, J.R., Nobre, M., (2009) Igualdade de direitos entre mulheres e homens, , (Coord.) Brasília: Ministério da Justiça SAL. (Projeto Pensando o Direito)Rodriguez, J.R., Nobre, M., (2010) Processo Legislativo e Controle de Constitucionalidade, , Brasília: Ministério da Justiça SAL. (Projeto Pensando o Direito

Diagnóstico do uso do fogo no entorno do Parque Estadual da Serra do Brigadeiro (PESB), MG Diagnosis of the use of fire at Parque Estadual da Serra do Brigadeiro (PESB), MG

O emprego do fogo é uma prática alternativa comum no meio rural, por ser uma técnica eficiente para diversas finalidades na visão de muitos agricultores. Esta técnica requer uma série de cuidados para não incorrer em desastres ambientais, como incêndios florestais. Os objetivos deste trabalho foram realizar um diagnóstico do uso do fogo pelos produtores rurais do entorno do Parque Estadual da Serra do Brigadeiro (PESB), Estado de Minas Gerais, e verificar sua percepção com relação aos incêndios florestais. Para tanto, foram amostradas três regiões distintas e representativas do entorno do PESB: Araponga, Pedra Bonita e Fervedouro. Os dados foram obtidos mediante entrevistas semi-estruturadas, com uso de questionário. Concluiu-se que o uso do fogo é ainda uma prática comum entre os produtores rurais, por ser mais viável economicamente e pelo seu rápido efeito. Entretanto, há um consenso sobre os prejuízos que ele pode causar ao solo, às suas vidas e ao meio ambiente.<br>The use of fire slash and Burn is a common alternative practice in the rural area, seen by many farmers as an efficient technique for various purposes. However, this technique should be carefully employed so as not to cause environmental disasters, such as forest fires. The objective of this work is to make a diagnosis of the use of fire by farmers nearby the Parque Estadual da Serra do Brigadeiro (PESB), in Minas Gerais and verify their perception of forest fire. The use of fire is still a common practice among farmers since it is economically viable and presents fast results. However, there is a consensus concerning its harmful effects on the soil, human life and the environment