Defects along the T(H)17 differentiation pathway underlie genetically distinct forms of the hyper IgE syndrome


AL KHATIB S., Keles S., GARCIA-LIORET M., Koc-Aydiner E. K. , Reisli I., Artac H., ...Daha Fazla

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, cilt.124, ss.342-348, 2009 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 124 Konu: 2
  • Basım Tarihi: 2009
  • Doi Numarası: 10.1016/j.jaci.2009.05.004
  • Dergi Adı: JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
  • Sayfa Sayıları: ss.342-348

Özet

Background: The hyper IgE syndrome (HIES) is characterized by abscesses, eczema, recurrent infections, skeletal and connective tissue abnormalities, elevated serum IgE, and diminished inflammatory responses. It exists as autosomal-dominant and autosomal-recessive forms that manifest common and distinguishing clinical features. A majority of those with autosomal-dominant HIES have heterozygous mutations in signal transducer and activator of transcription (STAT)-3 and impaired T(H)17 differentiation.