Source:http://linkedlifedata.com/resource/pubmed/id/14715622
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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
9
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pubmed:dateCreated |
2004-4-20
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pubmed:abstractText |
Children with the severe phenotype of the genetic immunodeficiency disease leukocyte adhesion deficiency or LAD experience life-threatening bacterial infections because of molecular defects in the leukocyte integrin CD18 molecule and the resultant failure to express the CD11/CD18 adhesion molecules on the leukocyte surface. Hematopoietic stem cell transplantation remains the only definitive therapy for LAD; however, the degree of donor chimerism and particularly the number of CD18(+) donor-derived neutrophils required to reverse the disease phenotype are not known. We performed nonmyeloablative hematopoietic stem cell transplantations from healthy matched littermates in 9 dogs with the canine form of LAD known as CLAD and demonstrate that in the 3 dogs with the lowest level of donor chimerism, less than 500 CD18(+) donor-derived neutrophils/microL in the peripheral blood of the CLAD recipients resulted in reversal of the CLAD disease phenotype. These results demonstrate the value of a disease-specific, large-animal model for identifying the lowest therapeutic level required for successful cellular and gene therapy.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
AIM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
May
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pubmed:issn |
0006-4971
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pubmed:author |
pubmed-author:BacherJohn DJD,
pubmed-author:BauerThomas RTRJr,
pubmed-author:BurkholderTanyaT,
pubmed-author:ColendaLynL,
pubmed-author:CreevyKate EKE,
pubmed-author:DonahueRobert ERE,
pubmed-author:EmbreeLisa JLJ,
pubmed-author:GuYu-chenYC,
pubmed-author:HicksteinDennis DDD,
pubmed-author:MetzgerMark EME,
pubmed-author:RominesChrisC,
pubmed-author:ThomasMarvin LML3rd,
pubmed-author:TuschongLaura MLM
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pubmed:issnType |
Print
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pubmed:day |
1
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pubmed:volume |
103
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
3582-9
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pubmed:meshHeading |
pubmed-meshheading:14715622-Animals,
pubmed-meshheading:14715622-Antigens, CD18,
pubmed-meshheading:14715622-DNA,
pubmed-meshheading:14715622-Dog Diseases,
pubmed-meshheading:14715622-Dogs,
pubmed-meshheading:14715622-Flow Cytometry,
pubmed-meshheading:14715622-Hematopoietic Stem Cell Transplantation,
pubmed-meshheading:14715622-Leukocyte Count,
pubmed-meshheading:14715622-Leukocyte-Adhesion Deficiency Syndrome,
pubmed-meshheading:14715622-Neutrophils,
pubmed-meshheading:14715622-Phenotype,
pubmed-meshheading:14715622-Transplantation, Homologous,
pubmed-meshheading:14715622-Transplantation Chimera,
pubmed-meshheading:14715622-Treatment Outcome
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pubmed:year |
2004
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pubmed:articleTitle |
Very low levels of donor CD18+ neutrophils following allogeneic hematopoietic stem cell transplantation reverse the disease phenotype in canine leukocyte adhesion deficiency.
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pubmed:affiliation |
Experimental Transplantation and Immunology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bldg 10, Rm 12C-116, 10 Center Dr, MSC1907, Bethesda, MD 20892-1907, USA. bauert@mail.nih.gov
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pubmed:publicationType |
Journal Article
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