@entity1117 ( @entity1117 ) is an @entity74 caused by mutations in one of the five genes encoding reduced @entity1110 oxidase subunits, which subsequently lead to impairment in the production of microbicidal reactive @entity26 species (ROS). In order to offer several cell line models of @entity1117 and therefore support research on pathophysiology and new therapeutic approaches, we optimized protocols to differentiate induced pluripotent stem cells (iPSCs) from wild-type, X(0)-, AR22(0)- and AR47(0)- @entity1117 @entity1 's fibroblasts into neutrophils and into macrophages. Aberrant genetic clones were discarded after chromosome karyotyping and array-comparative genomic hybridization analysis. All remaining iPSC lines showed @entity1 embryonic stem cell-like morphology, expressed all tested pluripotency markers and formed embryoid bodies that contained cells originating from all three primary germ layers. Furthermore, each @entity1117 @entity1 -specific iPSC line retained the gp91 (phox) , @entity1114 (phox) , and @entity9822 (phox) mutations found in the corresponding @entity1 's neutrophils. The average production of @entity2280 (+) progenitors was of 1.5*10(6) cells after 10 days of differentiation of 10*10(6) iPSCs. They were terminally differentiated into about 3*10(5) neutrophils or into 3*10(7) macrophages. Based on morphological, phenotypical, and functional criteria both phagocyte types were mature and indistinguishable from the native @entity1 neutrophils and macrophages. However, neutrophils and macrophages derived from X(0)-, AR22(0)-, and AR47(0)- @entity1117 @entity1 -specific iPSC lines lacked ROS production and the corresponding mutated proteins. To simplify the phagocytes' production upon request, progenitors can be cryopreserved. In conclusion, we describe a reproducible, simple, and efficient way to generate neutrophils and macrophages from iPSCs and provide a new cellular model for the AR22(0)- @entity1117 genetic form that has not been described before.

Optimized Generation of Functional Neutrophils and Macrophages from XXXX -Specific Induced Pluripotent Stem Cells: Ex Vivo Models of X(0)-Linked, AR22(0)- and AR47(0)- Chronic Granulomatous Diseases.

@entity1 :: ('9606', 'Species') :: ['human', 'patient'] @entity26 :: ('MESH:D010100', 'Chemical') :: ['oxygen'] @entity1110 :: ('MESH:D009249', 'Chemical') :: ['nicotinamide-adenine-dinucleotide-phosphate'] @entity1114 :: ('5341', 'Gene') :: ['p47'] @entity9822 :: ('11261', 'Gene') :: ['p22'] @entity74 :: ('MESH:D030342', 'Disease') :: ['inherited orphan disorder'] @entity2280 :: ('947', 'Gene') :: ['CD34'] @entity1117 :: ('MESH:D006105', 'Disease') :: ['Chronic granulomatous disease', 'CGD']
@entity1 :: (9606,Species) :: ['Patient']
