Mixed chimeric hematopoietic stem cell transplant reverses the disease phenotype in canine leukocyte adhesion deficiency.
Vet Immunol Immunopathol
; 95(3-4): 113-21, 2003 Oct 15.
Article
em En
| MEDLINE
| ID: mdl-12963272
ABSTRACT
The genetic disease canine leukocyte adhesion deficiency (CLAD) is characterized by recurrent, severe bacterial infections, typically culminating in death by 6 months of age. CLAD is due to a mutation in the leukocyte integrin CD18 subunit, which prevents surface expression of the CD11/CD18 leukocyte integrin complex. We demonstrate that stable mixed donorhost hematopoietic chimerism, achieved by a non-myeloablative bone marrow transplant from a histocompatible littermate, reverses the disease phenotype in CLAD. Donor chimerism following the transplant was demonstrated both by flow cytometric detection of donor-derived CD18-positive leukocytes in the peripheral blood of the recipient, and by the demonstration of donor-derived DNA microsatellite repeats in the peripheral blood leukocytes of the recipient. These results indicate that mixed hematopoietic chimerism reverses the clinical phenotype in CLAD and represents a potential therapeutic approach for the human disease leukocyte adhesion deficiency.
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Base de dados:
MEDLINE
Assunto principal:
Quimeras de Transplante
/
Síndrome da Aderência Leucocítica Deficitária
/
Transplante de Células-Tronco Hematopoéticas
/
Doenças do Cão
Idioma:
En
Ano de publicação:
2003
Tipo de documento:
Article