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

Al Khatib, Shadi; Keles, Sevgi; Garcia-Lioret, Maria; Koc-Aydiner, Elif; Reisli, Ismail; ARTAÇ, HASİBE; Camcioglu, Yildiz; Cokugras, Haluk; Somer, Ayper; KÜTÜKÇÜLER, NECİL; YILMAZ, MUSTAFA; Ikinciogullari, Aydan; Yegin, Olcay; Yueksek, Mutlu; Genel, Ferah; Kucukosmanoglu, Ercan; Baki, Ali; Bahceciler, Nerin; Rambhatla, Anupama; Nickerson, Derek; McGhee, Sean; Barlan, Isil; Chatila, Talal

doi:10.1016/j.jaci.2009.05.004

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., ARTAÇ H., ...Daha Fazla

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, cilt.124, sa.2, ss.342-348, 2009 (SCI-Expanded, Scopus)

Yayın Türü: Makale / Tam Makale
Cilt numarası: 124 Sayı: 2
Basım Tarihi: 2009
Doi Numarası: 10.1016/j.jaci.2009.05.004
Dergi Adı: JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
Sayfa Sayıları: ss.342-348
Çukurova Üniversitesi Adresli: Hayır

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.