Hereditary human complement C3 deficiency owing to reduced levels of C3 mRNA

Hereditary human complement C3 deficiency owing to reduced levels of C3 mRNA

Autor Ulbrich, Axel Gustavo Google Scholar
Florido, Marlene Pereira Carvalho Google Scholar
Nudelman, Victor Autor UNIFESP Google Scholar
Reis, Edmara da Silva Google Scholar
Baracho, Gisele Vanessa Google Scholar
Isaac, Lourdes Google Scholar
Instituição Universidade de São Paulo (USP)
Universidade Federal de São Paulo (UNIFESP)
Resumo An 8-year-old son (L.A.S.) of consanguineous parents, presented recurrent bacterial infections, vasculitis and extremely low levels of serum C3 (0.15 mug/ml). the classical and alternative pathway haemolytic activities and the generation of opsonins and chemotactic factors derived from the activation of the complement system were markedly affected in the proband's serum. An in vitro addition of purified C3 restored the classical pathway-dependent haemolytic activity of his serum. Autoradiographs of the proband's lipopolysaccharide (LPS)-stimulated and S-35-labelled fibroblast supernatants after that the SDS-PAGE revealed no C3 alpha or beta chains. the amount of C3 mRNA synthesized by the proband's fibroblasts, as evaluated by reverse transcription-polymerase chain reaction (RT-PCR) assays, was greatly reduced.
Idioma Inglês
Data de publicação 2001-06-01
Publicado em Scandinavian Journal of Immunology. Oxford: Blackwell Science Ltd, v. 53, n. 6, p. 622-626, 2001.
ISSN 0300-9475 (Sherpa/Romeo, fator de impacto)
Publicador Blackwell Science Ltd
Extensão 622-626
Direito de acesso Acesso aberto Open Access
Tipo Artigo
Web of Science WOS:000169483700013
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