@entity915 ( @entity915 ) is the most common form of @entity1 @entity915 and is caused by mutations in the common gamma chain (gammac), a shared component of the interleukin (IL)-2, @entity3646 , @entity6977 , @entity4666 , @entity8252 , and @entity15787 receptors. BMT for @entity1 @entity915 results in engraftment of donor T-cells and reconstitution of normal T-cell function but engraftment of few, if any, donor B-cells and poor reconstitution of humoral immune function. @entity235 @entity915 is also caused by mutations in the yc and has an immunological phenotype identical to that of @entity1 @entity915 . We have previously reported that transplantation of nonconditioned @entity915 @entity235 with unfractionated histocompatible bone marrow results in engraftment of both donor B- and T-cells and reconstitution of normal T-cell and humoral immune function. In this study, we assessed the ability of purified @entity235 @entity16129 + bone marrow cells to reconstitute lymphoid populations after histocompatible BMT in 6 nonablated @entity915 @entity235 . All @entity235 showed engraftment of donor T-cells, with T-cell regeneration occurring through a thymic-dependent pathway, and had reconstituted normal T-cell function. In contrast to our previous studies, only 3 @entity235 had engraftment of donor B-cells and reconstituted normal antigen-specific B-cell function post-BMT. The variable donor B-cell engraftment and reconstitution of normal humoral immune function observed in this study are similar to the outcomes observed in the majority of @entity1 @entity915 @entity1 following BMT. This study demonstrates that @entity235 @entity16129 + cells contain progenitors capable of immune reconstitution and is the first study to document the ability of @entity16129 + bone marrow cells to reconstitute normal B- and T-cell function in a nonablated large-animal model of BMT. This study also demonstrates that the quality of immune reconstitution following @entity16129 + BMT may be dosage dependent Thus @entity235 @entity915 provides a large-animal preclinical model that can be used not only to determine the optimal conditions for both donor B- and T-cell engraftment following @entity16129 BMT, but also to develop and evaluate strategies for gene therapy protocols that target @entity16129 cells.

Transplantation of X-linked severe combined immunodeficient @entity235 with XXXX + bone marrow cells.

@entity1 :: ('9606', 'Species') :: ['human', 'patients'] @entity4666 :: ('3578', 'Gene') :: ['IL-9'] @entity235 :: ('9615', 'Species') :: ['canine', 'Canine', 'dogs'] @entity6977 :: ('3574', 'Gene') :: ['IL-7'] @entity915 :: ('MESH:D053632', 'Disease') :: ['X-linked severe combined immunodeficiency', 'X-SCID', 'SCID'] @entity15787 :: ('59067', 'Gene') :: ['IL-21'] @entity8252 :: ('3600', 'Gene') :: ['IL-15'] @entity16129 :: ('415130(Tax:9615)', 'Gene') :: ['CD34'] @entity3646 :: ('3565', 'Gene') :: ['IL-4']
@entity16129 :: (415130(Tax:9615),Gene) :: ['CD34']
