Altered expression of neutrophil peripheral benzodiazepine receptor in
X-linked chronic granulomatous disease
F Zavala, F Veber and B Descamps-Latscha
INSERM U25, Hopital Necker, Paris, France.
This study was aimed at determining whether the peripheral benzodiazepine
receptor (PBZDR), which is abundantly expressed on mononuclear phagocytes,
is involved in host defense mechanisms depending on phagocyte
membrane-associated NADPH-oxidase complex. Analysis by reversible and
covalent binding of PBZDR expression on human neutrophils shows that it is
modulated during NADPH-oxidase activation with phorbol 12-myristate
13-acetate. Based on a series of 17 patients with chronic granulomatous
disease (CGD), results show that PBZDR expression is dramatically impaired
in X-linked CGD, an inherited disorder due to a mutation on the gene coding
for cytochrome b558 NADPH- oxidase component, whereas it is unaffected in
autosomal recessive CGD where cytochrome b558 is normally expressed,
suggesting a link between PBZDR and cytochrome b558 expressions. PBZDR can
be assigned by covalent binding to an 18-Kd membrane protein. These results
suggest that the neutrophil PBZDR, which can accommodate the widely
prescribed anxiolytic drug Valium (diazepam), is involved in host defense
against pathogens, a function that could be affected by neuroimmune
interactions.
Volume 76,
Issue 1,
pp. 184-188,
07/01/1990
Copyright © 1990 by The American Society of Hematology
