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PubMed · 1683141

Gene therapy for X-linked immunodeficiencies.

C Kinnon·M A O'Reilly·A Pelham·S Malcolm·C D Porter·M H Parkar·M K Collins·R J Leveinsky
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AgammaglobulinemiaCells, CulturedChromosome MappingDosage Compensation, GeneticElectrophoresis, Polyacrylamide GelGenes, RecessiveGenes, SyntheticGenetic LinkageGenetic MarkersGenetic TherapyGenetic VectorsHematopoietic Stem CellsHumansImmunologic Deficiency SyndromesPolymorphism, Restriction Fragment LengthPrenatal DiagnosisPromoter Regions, GeneticReceptors, Interleukin-2Recombinant Fusion ProteinsRetroviridaeTransfectionX ChromosomeJournal ArticleResearch Support, Non-U.S. Gov't

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C Kinnon, M A O'Reilly, A Pelham, S Malcolm, C D Porter, M H Parkar, M K Collins, R J Leveinsky. Gene therapy for X-linked immunodeficiencies.. https://pubmed.ncbi.nlm.nih.gov/1683141/

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Impaired Toll-like receptor 8-mediated IL-6 and TNF-alpha production in antigen-presenting cells from patients with X-linked agammaglobulinemia.

The critical role of Bruton tyrosine kinase (Btk) in B cells has been documented by the block of B-cell development in X-linked agammaglobulinemia (XLA). Less is known about Btk function in myeloid cells. Several pieces of evidence indicate that Btk is a component of Toll-like receptor (TLR) signaling. We analyzed whether Btk deficiency in XLA is associated with an impaired dendritic cell (DC) compartment or defective TLR signaling. We analyzed the expression of TLRs 1 to 9 on myeloid DCs generated from XLA patients and evaluated their response to activation by specific TLR agonists. We show that XLA patients have normal numbers of circulating DCs. Btk-deficient DCs have no defect in response to stimulation of TLRs 1/2, 2/6, 3, 4, and 5 but display a profound impairment of IL-6 and TNF-alpha production in response to stimulation by TLR-8 cognate agonist, ssRNA. These findings may provide an explanation for the susceptibility to enteroviral infections in XLA patients.

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