title: Bone marrow transplantation for canine X-linked severe combined immunodeficiency.
abstract: Canine X-linked severe combined immunodeficiency (XSCID) is due to mutations in the common gamma chain which is a subunit of the receptors of IL-2, IL-4, IL-7, IL-9 and IL-15. Bone marrow transplantation (BMT) of human XSCID patients without pretransplant conditioning (cytoablation) results in engraftment of donor T-cells and reconstitution of T-cell function but engraftment of few, if any, donor B cells with resultant poor reconstitution of humoral immune function. In this study, we show that XSCID dogs can be transplanted with allogeneic bone marrow cells resulting in engraftment of both donor B and T cells and reconstitution of full systemic immune function including normal humoral immune function without the need for cytoablation.
authors.affiliation: Department of Clinical Studies-Philadelphia, School of Veterinary Medicine, University of Pennsylvania 19104, USA. hartnett@vet.upenn.edu
authors.forename: B J
authors.initials: BJ
authors.lastname: Hartnett
authors.forename: P S
authors.initials: PS
authors.lastname: Henthorn
authors.forename: P F
authors.initials: PF
authors.lastname: Moore
authors.forename: K I
authors.initials: KI
authors.lastname: Weinberg
authors.forename: H D
authors.initials: HD
authors.lastname: Ochs
authors.forename: P J
authors.initials: PJ
authors.lastname: Felsburg
mesh_terms: D000818:Animals; D016026:Bone Marrow Transplantation / Q000662:veterinary*; D004195:Disease Models, Animal; D004283:Dog Diseases / Q000235:genetics / Q000628:therapy*; D004285:Dogs; D008040:Genetic Linkage; D016130:Immunophenotyping / Q000662:veterinary; D016511:Severe Combined Immunodeficiency / Q000235:genetics / Q000628:therapy / Q000662:veterinary*; D014960:X Chromosome