Cytochrome b and FAD content in polymorphonuclear leucocytes in a family with X-linked chronic granulomatous disease.
Scand J Haematol
; 37(4): 333-6, 1986 Oct.
Article
em En
| MEDLINE
| ID: mdl-3787183
ABSTRACT
Chronic granulomatous disease (CGD), an immunodeficiency syndrome characterized by extreme susceptibility to bacterial infections, is due to a defect of the respiratory burst in human phagocytes. NADPH oxidase, the enzyme that catalyzes the reduction of oxygen and the release of oxidative radicals, was studied in polymorphonuclear leucocytes (PMNs) in a family affected by an x-linked inheritance form at high penetrance of the disease. The contents of cytochrome b, suggested as the terminal component of the oxidase electron transport chain, and FAD, the hypothetical proximal component of the chain, were determined in patients and in carriers. Cytochrome b showed the typical behaviour of x-linked CGD total absence in patients, intermediate values in carriers. FAD content evaluated on plasma membranes was less decreased than cytochrome b. Carriers also showed a decrease of this flavoprotein. Cytochrome b and FAD contents were compared to NBT test and superoxide production a clear correlation was observed for the cytochrome b, but FAD plasma membrane evaluation could also be an interesting tool for the metabolic characterization of the disease in patients and in carriers.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Cromossomo X
/
Grupo dos Citocromos b
/
Flavina-Adenina Dinucleotídeo
/
Doença Granulomatosa Crônica
/
Ligação Genética
/
Neutrófilos
Limite:
Adult
/
Child
/
Female
/
Humans
/
Infant
/
Male
Idioma:
En
Revista:
Scand J Haematol
Ano de publicação:
1986
Tipo de documento:
Article
País de publicação:
DENMARK
/
DINAMARCA
/
DK