Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
3202702 | Journal of Allergy and Clinical Immunology | 2008 | 25 Pages |
Abstract
Distinct NEMO hypomorphs define specific disease and genetic characteristics. A reconstitution system can identify attributes of hypomorphisms independent of an individual's genetic background. Apoptosis susceptibility in L153R reconstituted cells defines a specific phenotype of this mutation that likely contributes to the excessive inflammation with which it is clinically associated.
Keywords
IKKNuclear factor-κB essential modulator7-Amino actinomycin DGenetic database7-AADA20NF-κBNEMOTCrGFPEDAFACSIκB kinaseElectrophoretic mobility shift assayfluorescence-activated cell sortingDendritic cellEMSA یا electrophoretic mobility shift assay nuclear factor-κBNF-κB activationgreen fluorescent proteinImmunodeficiencyT-cell receptor
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Authors
Eric P. MD, Linda BA, Laura A. BS, Lisa A. PhD, Pinaki P. PhD, Michael J. PhD, Jordan S. MD, PhD,