The hyper-IgE syndromes lessons in nature, from bench to bedside 英文参考文献.docVIP

REVIEWARTICLE TheHyper-IgESyndromes:LessonsinNature,FromBench toBedside EfrenL.Rael,MD,1RobertT.Marshall,BS,2andJonathanJ.McClain,MS2 DNA-binding domains (important for transcriptional regula- Abstract: Hyper-IgE syndrome is a primary immunode?ciency tion),theSrchomology2(SH2)domains(importantinStat protein dimerization and receptor contacts), and the gene markedbyabnormalitiesinthecoordinationofcell–cellsignalingwith the potential to affect TH17 cell, B cell, and neutrophil responses. splice sites/trans activation sites (associated with protein Clinical manifestations include recurrent ski