FATAL REDUCING BODY MYOPATHY - ULTRASTRUCTURAL and IMMUNOHISTOCHEMICAL OBSERVATIONS

FATAL REDUCING BODY MYOPATHY - ULTRASTRUCTURAL and IMMUNOHISTOCHEMICAL OBSERVATIONS

Autor Kiyomoto, B. H. Google Scholar
Murakami, N. Google Scholar
Kobayashi, Y. Google Scholar
Nihei, K. Google Scholar
Tanaka, T. Google Scholar
Takeshita, K. Google Scholar
NONAKA, I Google Scholar
Instituição NATL CHILDRENS HOSP
TOTTORI UNIV
NATL CTR NEUROL & PSYCHIAT
Universidade Federal de São Paulo (UNIFESP)
Resumo Two female infants who developed normally during infancy began to have progressive muscle hypotonia and weakness from 2 year 10 months and 2 years 3 months of ages, respectively. Both patients had rapidly progressive muscle weakness with death from respiratory failure at 4 years 11 months and 3 years 9 months, respectively. in addition to mild inflammation in their muscle biopsies, the most striking finding was the presence of numerous reducing bodies (RB) in almost all degenerating fibers. By electron microscopy, these bodies consisted of fine granular material, usually located around the degenerating nucleus. These bodies showed no immunohistochemical reaction to antibodies against structural, cytoskeletal and membrane proteins and a histone-specific antibody against nuclei and chromosomes. They were occasionally positively stained with a ubiquitin antibody. Although the origin of these bodies remains unknown, they appeared to be related to active myofibrillar degeneration, probably resulting form primary nuclear degeneration.
Palavra-chave CONGENITAL MYOPATHY
REDUCING BODY MYOPATHY
IMMUNOHISTOCHEMISTRY
ULTRASTRUCTURE
RIMMED VACUOLE
Idioma Inglês
Data de publicação 1995-01-01
Publicado em Journal of the Neurological Sciences. Amsterdam: Elsevier B.V., v. 128, n. 1, p. 58-65, 1995.
ISSN 0022-510X (Sherpa/Romeo, fator de impacto)
Publicador Elsevier B.V.
Extensão 58-65
Fonte http://dx.doi.org/10.1016/0022-510X(94)00204-2
Direito de acesso Acesso restrito
Tipo Artigo
Web of Science WOS:A1995QD06900008
Endereço permanente http://repositorio.unifesp.br/handle/11600/25453

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