RESUMO
Neurofibromatosis Type I (NF1) is a rare genetic disorder. NF1 patients frequently develop a benign tumor in peripheral nerve plexuses called plexiform neurofibroma. In the past two decades, tissue-specific Nf1 knockout mouse models were developed using commercially available tissue-specific Cre recombinase and the Nf1 flox mice to mimic neurofibroma development. However, these models develop para-spinal neurofibroma, recapitulating a rare type of neurofibroma found in NF1 patients. The NPcis mouse model developed a malignant version of neurofibroma called malignant peripheral nerve sheath tumor (MPNST) within 3 to 6 months but intriguingly without apparent benign precursor lesion. Here, we revisited the NPcis model and discovered that about 20% display clinical signs similar to Nf1 tissue-specific knockout mice models. However, a systematic histological analysis could not explain the clinical signs we observed although we noticed lesions reminiscent of a neurofibroma in a peripheral nerve, a cutaneous neurofibroma, and para-spinal neurofibroma on rare occasions in NPcis mice. We also observed that 10% of the mice developed a malignant peripheral nerve sheath tumor (MPNST) spontaneously, coinciding with their earring tag identification. Strikingly, half of the sciatic nerves from NPcis mice developed plexiform neurofibroma within 1-6 months when intentionally injured. Thus, we provided a procedure to turn the widely used NPcis sarcoma model into a model recapitulating plexiform neurofibroma.
Assuntos
Modelos Animais de Doenças , Neurofibroma Plexiforme , Animais , Neurofibroma Plexiforme/patologia , Camundongos , Nervo Isquiático/patologia , Camundongos Knockout , Neurofibromatose 1/patologia , Neurofibromatose 1/genética , Neurofibromina 1/genéticaRESUMO
Três temas ocupam o espaço desta apresentação. Os primeiros dois debruçam-se na mesma problemática, mas sob um ângulo diferente. A relação entre o bem pessoal e o bem comum pode efectivamente ser objecto de pelo menos dois discursos diferentes, o da ética e o do direito. Além disso, poderá aparecer estranho ligar a estes dois temas um terceiro, reservado à objecção de consciência. Mas este tem cabimento na medida em que constitui, ao nível da cidadania, uma forma sociopolítica de tensão entre um bem pessoal e um bem comum, de natureza social ou política. Desta maneira, ética, direito e cidadania política interferem quando queremos abrir o processo das relações entre o bem pessoal e o bem comum. O tratamento dos problemas da pandemia não escapou sempre a tais questões espinhosas.
Three themes occupy the space of this presentation. The first two deal with the same problem, but from a different angle. The relationship between the personal good and the common good can in fact be the subject of at least two different discourses, that of ethics -and that of law. In addition, it may seem strange to link these two themes to a third, reserved for conscientious objection. However, this is valid in that it constitutes, at the level of citizenship, a socio-political form of tension between a personal and a common good, of a social or political nature. In this way, ethics, law and political citizenship interfere when we want to open the process of relations between the personal good and the common good. The treatment of pandemic problems has not always escaped such thorny issues.
Tres temas ocupan el espacio de esta presentación. Los dos primeros abordan el mismo problema, pero desde un ángulo diferente. La relación entre el bien personal y el bien común puede de hecho ser objeto de al menos dos discursos diferentes, el de la ética y el del derecho. Además, puede parecer extraño vincular estos dos temas con un tercero, reservado a la objeción de conciencia. Sin embargo, esto es válido porque constituye, a nivel de ciudadanía, una forma sociopolítica de tensión entre un bien personal y un bien común, de carácter social o político. De esta forma, la ética, el derecho y la ciudadanía política interfieren cuando queremos abrir el proceso de relaciones entre el bien personal y el bien común. El tratamiento de los problemas pandémicos no siempre ha escapado a cuestiones tan espinosas.
