@entity17454 ( @entity17454 ) is an @entity1112 characterized by @entity882 and progressive deterioration of the immune function. Dendritic cells (DC) are key effectors in the induction of specific immunity and are highly specialized in antigen uptake and subsequent migration to draining lymph nodes. DC were generated in vitro from circulating monocytes from ten @entity17454 @entity1 characterized by a different disease score. Immature DC showed similar morphology and membrane phenotype, as compared to normal DC. In chemotaxis assay, immature DC had a reduced migration in response to @entity10557 / @entity10557 , but efficiently endocytosed the macromolecules @entity2823 -dextran and FITC-albumin. Upon terminal differentiation with @entity558 or @entity2936 ligand, the acquisition of a mature surface phenotype was variably achieved among @entity17454 @entity1 , with increased expression of @entity7938 , @entity2937 and @entity5687 . In contrast, the expression of @entity8730 was usually low. A defective up-regulation of @entity8730 was also observed in the lymph node from one @entity17454 @entity1 , whose DC stained positively for @entity5687 . Mature DC from all the @entity1 tested, but one, significantly migrated in vitro in response to @entity11274 , a finding confirmed in vivo by the detection of HLA-DR/DC LAMP-positive cells in secondary lymphoid organs. When tested in MLR assays, both immature and mature @entity17454 DC induced allogenic T cell proliferation in a manner comparable to control DC. Collectively these results suggest that, although many functional activities of @entity17454 DC are essentially similar to normal DC, subtle and selective alterations of DC differentiation were also observed, with reduced migratory activity of immature DC and defective @entity8730 expression upon maturation.

Monocytes from Wiskott-Aldrich XXXX differentiate in functional mature dendritic cells with a defect in @entity8730 expression.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity558 :: ('MESH:C536528', 'Disease') :: ['LPS'] @entity2937 :: ('942', 'Gene') :: ['CD86'] @entity882 :: ('MESH:D013921', 'Disease') :: ['congenital thrombocytopenia'] @entity17454 :: ('MESH:D014923', 'Disease') :: ['WAS', 'Wiskott-Aldrich syndrome'] @entity1112 :: ('MESH:D040181', 'Disease') :: ['X-linked disorder'] @entity2936 :: ('958', 'Gene') :: ['CD40'] @entity7938 :: ('941', 'Gene') :: ['CD80'] @entity8730 :: ('9308', 'Gene') :: ['CD83'] @entity2823 :: ('MESH:D016650', 'Chemical') :: ['FITC'] @entity5687 :: ('27074', 'Gene') :: ['DC-LAMP'] @entity11274 :: ('6363', 'Gene') :: ['MIP-3beta'] @entity10557 :: ('6348', 'Gene') :: ['MIP-1alpha', 'CCL3']
@entity1 :: (9606,Species) :: ['patients']
