Switch to
| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
10
|
| pubmed:dateCreated |
1994-12-20
|
| pubmed:abstractText |
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.
|
| pubmed:grant | |
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
AIM
|
| pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/CYBB protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Cytochromes b5,
http://linkedlifedata.com/resource/pubmed/chemical/Membrane Glycoproteins,
http://linkedlifedata.com/resource/pubmed/chemical/NADPH Oxidase,
http://linkedlifedata.com/resource/pubmed/chemical/Superoxides
|
| pubmed:status |
MEDLINE
|
| pubmed:month |
Nov
|
| pubmed:issn |
0006-4971
|
| pubmed:author | |
| pubmed:issnType |
Print
|
| pubmed:day |
15
|
| pubmed:volume |
84
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
3311-6
|
| pubmed:dateRevised |
2007-11-14
|
| pubmed:meshHeading |
pubmed-meshheading:7949086-Blotting, Northern,
pubmed-meshheading:7949086-Cell Differentiation,
pubmed-meshheading:7949086-Cell Line,
pubmed-meshheading:7949086-Cell Membrane,
pubmed-meshheading:7949086-Clone Cells,
pubmed-meshheading:7949086-Cytochromes b5,
pubmed-meshheading:7949086-Genetic Vectors,
pubmed-meshheading:7949086-Granulocytes,
pubmed-meshheading:7949086-Granulomatous Disease, Chronic,
pubmed-meshheading:7949086-Humans,
pubmed-meshheading:7949086-Membrane Glycoproteins,
pubmed-meshheading:7949086-Mutation,
pubmed-meshheading:7949086-NADPH Oxidase,
pubmed-meshheading:7949086-Respiratory Burst,
pubmed-meshheading:7949086-Retroviridae,
pubmed-meshheading:7949086-Superoxides,
pubmed-meshheading:7949086-Transfection,
pubmed-meshheading:7949086-X Chromosome
|
| pubmed:year |
1994
|
| pubmed:articleTitle |
Retrovirus-mediated reconstitution of respiratory burst activity in X-linked chronic granulomatous disease cells.
|
| pubmed:affiliation |
Department of Pediatrics, Herman B Wells Center for Pediatric Research, James Whitcomb Riley Hospital for Children, Indiana University Medical Center, Indianapolis 46202-5225.
|
| pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
|