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Retrovirus-mediated reconstitution of respiratory burst activity in X-
linked chronic granulomatous disease cells
A Kume and MC Dinauer
Department of Pediatrics, Herman B Wells Center for Pediatric Research,
James Whitcomb Riley Hospital for Children, Indiana University Medical
Center, Indianapolis 46202-5225.
X-linked chronic granulomatous disease (X-CGD) results from mutations in
the gene encoding gp91phox, the larger subunit of the respiratory burst
oxidase cytochrome b. In this study, a recombinant retrovirus vector was
constructed and evaluated for its expression of human gp91phox in a human
X-CGD myeloid cell line in which the endogenous gp91phox gene had been
disrupted by gene targeting. The retrovirus construct, Zip/PGKgp91, was
first introduced into the GP+envAm12 amphotropic packaging line and yielded
virus producer clones with estimated titers of up to 1 x 10(5) cfu/mL.
Coculture infection of X- CGD myeloid cells with Zip/PGKgp91 resulted in
restoration of respiratory burst activity to 15% of the cells. Isolated
clonal infectants expressed relatively low levels of recombinant gp91phox
(< or = 12% of wild-type), but exhibited considerable superoxide-
generating activity (up to nearly 60% of wild-type). These results show the
feasibility of phenotypic correction of CGD using gene replacement therapy
and suggest that even modest levels of gp91phox expression may lead to
considerable functional correction of X-CGD neutrophils.
Volume 84,
Issue 10,
pp. 3311-3316,
11/15/1994
Copyright © 1994 by The American Society of Hematology
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