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Blood, 15 June 2001, Vol. 97, No. 12, pp. 3738-3745
GENE THERAPY
Variable correction of host defense following gene transfer and
bone marrow transplantation in murine X-linked chronic
granulomatous disease
Mary C. Dinauer,
Mary A. Gifford,
Nancy Pech,
Ling Lin Li, and
Patricia Emshwiller
From the Herman B Wells Center for Pediatric Research,
Departments of Pediatrics (Hematology/Oncology) and Medical and
Molecular Genetics, James Whitcomb Riley Hospital for Children, Indiana
University School of Medicine, Indianapolis, IN.
Chronic granulomatous disease (CGD) is an inherited
immunodeficiency in which the absence of the phagocyte
superoxide-generating nicotinamide adenine dinucleotide phosphate
(NADPH) oxidase results in recurrent bacterial and fungal infections. A
murine model of X-linked CGD (X-CGD) was used to explore variables
influencing reconstitution of host defense following bone marrow
transplantation and retroviral-mediated gene transfer. The
outcomes of experimental infection with Aspergillus
fumigatus, Staphylococcus aureus, or Burkholderia cepacia were compared in wild-type, X-CGD
mice, and transplanted X-CGD mice that were chimeric for either
wild-type neutrophils or neutrophils with partial correction of NADPH
oxidase activity after retroviral-mediated gene transfer. Host defense to these pathogens was improved in X-CGD mice even with correction of a
limited number of neutrophils. However, intact protection against
bacterial pathogens required relatively greater numbers of
oxidant-generating phagocytes compared to protection against A
fumigatus. The host response also appeared to be influenced by
the relative level of cellular NADPH oxidase activity, particularly for
A fumigatus. These results may have implications for
developing effective approaches for gene therapy of CGD.
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