Bone marrow transplantation in @entity1 @entity915 ( @entity915 ) without pretransplant conditioning results in engraftment of donor T cells and reconstitution of T-cell function but engraftment of few, if any, donor B cells and poor reconstitution of humoral immune function. Since bone marrow transplantation remains the most effective treatment of @entity915 @entity1 , better strategies are necessary to achieve optimum long-term results. @entity235 @entity915 , like @entity1 @entity915 , is due to mutations in the @entity16409 ( @entity16409 ) gene and has clinical and immunologic features identical to those of @entity1 @entity915 , making it a true homolog of the @entity1 disease. We have successfully performed bone marrow transplantation in three @entity915 @entity235 without pretransplant conditioning, using untreated bone marrow cells from mixed lymphocyte culture-nonreactive normal littermates. Unlike the experience in @entity1 @entity915 @entity1 , all three @entity235 engrafted both donor B and T cells and attained full reconstitution of immunologic function. Normal percentages of T cells and T-cell mitogenic responses were attained by 3 months posttransplant. CD3+ T cells after transplantation expressed the @entity1195 RA isoform indicating that the cells were recent thymic emigrants derived from immature progenitors. Serum IgG levels were within normal range by 5 months posttransplant. Immunization with the T-dependent antigen, bacteriophage phiX174, demonstrated normal antibody titers, immunologic memory, and class-switching. Polymerase chain reaction (PCR) analysis of the @entity16409 locus showed that 100% of circulating T cells and 30% to 50% of circulating B cells were donor-derived. None of the @entity235 developed clinically evident @entity2132 ( @entity2132 ). Thus, @entity235 @entity915 provides a model to determine the optimal conditions for bone marrow transplantation in @entity1 @entity1 , and to develop and test strategies for somatic gene therapy.

Full immunologic reconstitution following nonconditioned bone marrow transplantation for XXXX @entity915 .

@entity1 :: ('9606', 'Species') :: ['human', 'patients'] @entity235 :: ('9615', 'Species') :: ['canine', 'Canine', 'dogs'] @entity1195 :: ('5788', 'Gene') :: ['CD45'] @entity915 :: ('MESH:D053632', 'Disease') :: ['X-linked severe combined immunodeficiency', 'XSCID'] @entity2132 :: ('MESH:D006086', 'Disease') :: ['GVHD', 'graft-versus-host disease'] @entity16409 :: ('3561', 'Gene') :: ['gamma c', 'common gamma chain']
@entity235 :: (9615,Species) :: ['canine']
