Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học quốc tế cung cấp cho các bạn kiến thức về ngành y đề tài: Neuronal degeneration in autonomic nervous system of Dystonia musculorum mice | Tseng et al. Journal of Biomedical Science 2011 18 9 http content 18 1 9 JOURNAL OF BIOMEDICAL SCIENCE tì NSC The cost of publication in Journal of Biomedical Science Is borne by the National Science Council Taiwan RESEARCH Open Access Neuronal degeneration in autonomic nervous system of Dystonia musculorum mice Kuang-Wen Tseng 1 Mei-Lin Peng2 Yang-Cheng Wen1 Kang-Jen Liu1 Chung-Liang Chien3 Abstract Background Dystonia musculorum dt is an autosomal recessive hereditary neuropathy with a characteristic uncoordinated movement and is caused by a defect in the bullous pemphigoid antigen 1 BPAG1 gene. The neural isoform of BPAG1 is expressed in various neurons including those in the central and peripheral nerve systems of mice. However most previous studies on neuronal degeneration in BPAG1-deficient mice focused on peripheral sensory neurons and only limited investigation of the autonomic system has been conducted. Methods In this study patterns of nerve innervation in cutaneous and iridial tissues were examined using general neuronal marker protein gene product via immunohistochemistry. To perform quantitative analysis of the autonomic neuronal number neurons within the lumbar sympathetic and parasympathetic ciliary ganglia were calculated. In addition autonomic neurons were cultured from embryonic dt dt mutants to elucidate degenerative patterns in vitro. Distribution patterns of neuronal intermediate filaments in cultured autonomic neurons were thoroughly studied under immunocytochemistry and conventional electron microscopy. Results Our immunohistochemistry results indicate that peripheral sensory nerves and autonomic innervation of sweat glands and irises dominated degeneration in dt dt mice. Quantitative results confirmed that the number of neurons was significantly decreased in the lumbar sympathetic ganglia as well as in the parasympathetic ciliary ganglia of dt dt mice compared with those of wild-type mice. We also observed that the .
