Canine leukocyte adhesion deficiency colony for investigation of novel hematopoietic therapies

The genetic immunodeficiency disease canine leukocyte adhesion deficiency (CLAD) was originally described in juvenile Irish Setters with severe, recurrent bacterial infections. CLAD was subsequently shown to result from a mutation in the leukocyte integrin CD18 subunit which prevents leukocyte surfa...

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Published inVeterinary immunology and immunopathology Vol. 94; no. 1-2; pp. 11 - 22
Main Authors Creevy, Kate E, Bauer, Thomas R, Tuschong, Laura M, Embree, Lisa J, Colenda, Lyn, Cogan, Kevin, Starost, Matthew F, Haskins, Mark E, Hickstein, Dennis D
Format Journal Article
LanguageEnglish
Published Netherlands Elsevier B.V 15.07.2003
Subjects
Adhesion
Animals
Breeding
Canine
CD18 Antigens - analysis
Dog Diseases - genetics
Dog Diseases - pathology
Dog Diseases - therapy
Dogs - genetics
Female
Genotype
Heterozygote
Leukocyte
Leukocyte-Adhesion Deficiency Syndrome - genetics
Leukocyte-Adhesion Deficiency Syndrome - pathology
Leukocyte-Adhesion Deficiency Syndrome - therapy
Leukocyte-Adhesion Deficiency Syndrome - veterinary
Male
Minisatellite Repeats - genetics
Mutation - genetics
Pedigree
Phenotype
CLAD
Leukocyte
DLA
FACS
HOD
Adhesion
LAD
VNTR
Canine
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Summary:The genetic immunodeficiency disease canine leukocyte adhesion deficiency (CLAD) was originally described in juvenile Irish Setters with severe, recurrent bacterial infections. CLAD was subsequently shown to result from a mutation in the leukocyte integrin CD18 subunit which prevents leukocyte surface expression of the CD11/CD18 complex. We describe the development of a mixed-breed CLAD colony with clinical features that closely parallel those described in Irish Setters. We demonstrate that the early identification of CLAD heterozygotes and CLAD-affected dogs by a combination of flow cytometry and DNA sequencing allows the CLAD-affected animals to receive life-saving antibiotic therapy. The distinct clinical phenotype in CLAD, the ability to detect CD18 on the leukocyte surface by flow cytometry, and the history of the canine model in marrow transplantation, enable CLAD to serve as an attractive large-animal model for the investigation of novel hematopoietic stem cell and gene therapy strategies.
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ISSN:0165-2427
1873-2534
DOI:10.1016/S0165-2427(03)00057-6