Complete and partial deficiencies of complement factor D in a Dutch family.
J Clin Invest
; 84(6): 1957-61, 1989 Dec.
Article
em En
| MEDLINE
| ID: mdl-2687330
ABSTRACT
A young man suffering from recurrent Neisseria infections was shown to lack detectable serum complement factor D hemolytic activity. Addition to the patient's serum of purified factor D to a final concentration of 1 microgram/ml resulted in full restoration of the activity of the alternative pathway. Using an enzyme-linked immunosorbent assay, it was shown that the patient's serum did not contain measurable amounts of factor D antigen either. The sister, the father, as well as the parents of the mother had factor D levels within the normal range, and the factor D level of the mother was decreased. The capacity of the patient's serum, at concentrations up to 5%, to promote phagocytosis of Escherichia coli by normal human granulocytes was low when compared to normal serum. Substitution of the patient's serum with purified factor D resulted in a full restoration of opsonic activity. This study describes the first complete deficiency of factor D, and demonstrates its possible relation to recurrent Neisseria infections.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Enzimas Ativadoras do Complemento
/
Fator D do Complemento
/
Gonorreia
/
Meningite Meningocócica
Tipo de estudo:
Etiology_studies
Limite:
Adult
/
Humans
/
Male
Idioma:
En
Revista:
J Clin Invest
Ano de publicação:
1989
Tipo de documento:
Article
País de afiliação:
Holanda