Spontaneous Murine Neuroaxonal Dystrophy: a Model of Infantile Neuroaxonal Dystrophy
نویسندگان
چکیده
منابع مشابه
Infantile neuroaxonal dystrophy.
as encephalitis, diabetes, heart failure, carbon monoxide poisoning, and cerebral arteriosclerosis. However, in a few recorded cases spheroids were sufficiently numerous to dominate the morphological picture. Some of these patients were infants or children showing a somewhat uniform clinical picture and pattern of encephalopathy, so that they could have been cases of the same disease or syndrom...
متن کاملInfantile Neuroaxonal Dystrophy
Keywords Disease names and synonyms Definition/Diagnostic criteria Differential diagnosis Etiology Clinical description Diagnostic methods Epidemiology Management Unresolved questions References Abstract Infantile neuroaxonal dystrophy (INAD) is a rare autosomal recessive neurodegenerative disorder with onset in the first or second year of life. Frequency is unknown. It is characterized by a pr...
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Breath-holding spells are episodes of brief, involuntary cessations of breathing that occur in children in response to stimuli such as fear or injury. They are usually benign, and although very rarely, breath-holding spells accompany deteriorative neurological diseases. Here, we present two patients having infantile neuroaxonal dystrophy and breath-holding spells. Presence of the common respira...
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Mitochondrial dysfunction is increasingly being recognized as a therapeutic target in PLA26G-associated neurodegeneration (PLAN). We demonstrated in Drosophila and in human PLA2G6 mutant fibroblasts that loss of normal PLA2G6 activity is associated with increased mitochondrial lipid peroxidation and mitochondrial dysfunction. Furthermore, we identified the therapeutic benefits of deuterated pol...
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Neuritic degeneration is a prominent ultrastructural feature of scrapie in hamsters. To investigate the morphogenesis of neuritic degeneration, we examined brain tissues from hamsters infected with the 263K strain of scrapie virus and from age-matched controls at varying intervals following intracerebral inoculation. Dystrophic neurites--defined as dendrites, axonal preterminals, and myelinated...
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ژورنال
عنوان ژورنال: Journal of Comparative Pathology
سال: 2006
ISSN: 0021-9975
DOI: 10.1016/j.jcpa.2005.10.002