Autonomic nervous system involvement in the giant axonal neuropathy (GAN) KO mouse: implications for human disease

PURPOSE: Giant axonal neuropathy (GAN) is an inherited severe sensorimotor neuropathy. The aim of this research was to investigate the neuropathologic features and clinical autonomic nervous system (ANS) phenotype in two GAN knockout (KO) mouse models. Little is known about ANS involvement in GAN in humans, but autonomic signs and symptoms are commonly reported in early childhood. METHODS: Routine histology and immunohistochemistry was performed on GAN KO mouse specimens taken at various ages. Enteric dysfunction was assessed by quantifying the frequency, weight, and water content of defecation in GAN KO mice. RESULTS: Histological examination of the enteric, parasympathetic and sympathetic ANS of GAN KO mice revealed pronounced and widespread neuronal perikaryal intermediate filament inclusions. These neuronal inclusions served as an easily identifiable, early marker of GAN in young GAN KO mice. Functional studies identified an age-dependent alteration in fecal weight and defecation frequency in GAN KO mice. CONCLUSIONS: For the first time in the GAN KO mouse model, we described the early, pronounced and widespread neuropathologic features involving the ANS. In addition, we provided evidence for a clinical autonomic phenotype in GAN KO mice, reflected in abnormal gastrointestinal function. These findings in GAN KO mice suggest that consideration should be given to ANS involvement in human GAN, especially when considering treatments and patient care

Griaule, la etnografía del secreto

La Misión Dacar-Yibutí bajo el mando de Marcel Griaule inauguró la etnografía francesa de una manera moderna, espectacular, vinculándose a la última onda intelectual, artística y hasta deportiva. La producción que de allí ha surgido es enorme y de diverso calibre: por un lado, la obra de Leiris sobre la lengua secreta dogon y sobre un culto de posesión en Etiopía. Por el otro, la gran saga griaulista que construye un gigantesco sistema cosmológico en base a declaraciones muy sobreinterpretadas de un puñado de informantes. Tal vez el punto clave de esta última corriente sea la suposición de que su tarea es la revelación de un secreto. Es sobre "secreto" que este texto indaga.<br>The Dacar-Yibutí Mission under Marcel Griaule´s guidance has inaugurated French ethnography in a modern, spectacular fashion, connecting it to the latest intellectual, artistic, and even sports fashion. It has provoked the issuing of a great number of texts of distinct caliber: from one hand, the work of Leiris on the dogon language as well as on a possession cult from Ethiopia; on the other hand, the great griaulist saga which constructs a gigantic cosmological system based on overinterpretations of a handful number of informants' declarations. Perhaps the main point of this last trend is the premise that his task is to reveal a secret. This article inquiries on this "secret"