NEMO/IKKγ:: Linking NF-κB to human disease

被引:60
作者
Courtois, G [1 ]
Smahi, A
Israël, A
机构
[1] Inst Pasteur, CNRS, URA 1773, Unite Biol Mol Express Genique, Paris, France
[2] Hop Necker Enfants Malad, INSERM, Unite Rech Handicaps Genet Enfant, U393, Paris, France
关键词
D O I
10.1016/S1471-4914(01)02154-2
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Until recently, no genetic disease caused by NF-kappaB dysfunction was known. This changed with the identification of the X-linked gene encoding a molecule of the NF-kappaB signaling pathway, NEMO/IKK gamma Two distinct X-linked human diseases, incontinentia pigmenti (IP) and anhidrotic ectodermal dysplasia associated with immunodeficiency (EDA-ID), have been linked to NEMO/IKK gamma dysfunction, providing a unique view of the role that NF-kappaB plays in human development, skin homeostasis and innate and acquired immunity.
引用
收藏
页码:427 / 430
页数:4
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