Neuronal degeneration in autonomic nervous system of Dystonia musculorum mice
نویسندگان
چکیده
منابع مشابه
Motor Unit Abnormalities in Dystonia musculorum Mice
Dystonia musculorum (dt) is a mouse inherited sensory neuropathy caused by mutations in the dystonin gene. While the primary pathology lies in the sensory neurons of dt mice, the overt movement disorder suggests motor neurons may also be affected. Here, we report on the contribution of motor neurons to the pathology in dt(27J) mice. Phenotypic dt(27J) mice display reduced alpha motor neuron cel...
متن کاملA Cellular Mechanism of Neuronal Loss in the Dorsal Root Ganglia of Dystonia musculorum (dt) Mice
Dystonia musculorum (dt) is a mutant mouse with hereditary sensory neuropathy, and a defective bullous pemphigoid antigen 1 (BPAG1) gene is responsible for this mutation. In present study, we examined the distribution of neuronal intermediate filament proteins in the central and peripheral processes of the dorsal root ganglia (DRG) in adult dt mice by different approaches. We found that not onl...
متن کاملRe: "A possible cellular mechanism of neuronal loss in the dorsal root ganglia of dystonia musculorum (dt) mice".
Dystonia musculorum (dt) is a mutant mouse with hereditary sensory neuropathy. A defective bullous pemphigoid antigen 1 (BPAG1) gene is responsible for this mutation. In the present study, we examined the distribution of neuronal intermediate filament proteins in the central and peripheral processes of the dorsal root ganglia (DRG) in adult dt mice using different approaches. We found that not ...
متن کاملDevelopmental expression of BPAG1-n: insights into the spastic ataxia and gross neurologic degeneration in dystonia musculorum mice.
Ablation of the BPAG1 gene results in the dystonia musculorum mouse, exhibiting rapid spinal nerve degeneration, dystonic movements, and severe ataxia. By defining the developmental and tissue-specific expression of the neuronal form of BPAG1 (BPAG1-n) and by comparing the corresponding pathology in BPAG1 null mice, we seek here to understand how absence of BPAG1 results in this devastating phe...
متن کاملAssessment of autonomic nervous system function in patients with non-alcoholic cirrhosis
Automatic dysfunction may contribute to the hemodynamic disturbances in cirrhosis. Tests of autonomic function were performed in 31 patients with non-alcoholic cirrhosis and 30 healthy subjects. Patients were divided into 3 groups according to child- Pugh,s classification ( A.B.C). The patients with moderate and severe cirrhosis( child B,C) had significant parasympathetic disturbance ( in the v...
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ژورنال
عنوان ژورنال: Journal of Biomedical Science
سال: 2011
ISSN: 1423-0127
DOI: 10.1186/1423-0127-18-9