Vol.23 No.2

Review Article

IKZF1: a critical role in the pathogenesis of systemic lupus erythematosus?

Authors

Su-juan Hu1,2 , Lei-lei Wen1,2 , Xin Hu1,2 , Xian-yong Yin1,2 , Yong Cui1,2 , Sen Yang1,2 , Xue-jun Zhang1,2

  • Institute of Dermatology and Department of Dermatology, First Hospital of Anhui Medical University, 81 Meishan Road, Hefei, Anhui, 230032, People’s Republic of China
  • Key Laboratory of Dermatology of Ministry of Education, Hefei, People’s Republic of China
Received:

27 January 2012

Accepted:

12 June 2012

Published online:

11 July 2012

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Abstract

Ikaros family zinc finger 1, encoded by IKZF1, are lymphoid-restricted zinc finger transcription factors that share common N-terminal Kruppel-like zinc finger DNA-binding domain. IKZF1 play multiple important roles on regulators of lymphocyte differentiation and hematological tumor suppressor. Our genome-wide association (GWA) studies in systemic lupus erythematosus (SLE) independently identified genetic variants in IKZF1 associated with SLE, which are supported by other studies. Previous studies found that lower expression of IKZF1 may play critical roles in activating some signal pathways involved in SLE, such as signal transducers and activators of transcription (STAT)4 and interferon pathways. In addition, IKZF1 has been implicated in roles involved in some hematologic traits or abnormalities, such as erythrocyte measures, myelofibrosis, and acute lymphoblastic leukemia (ALL), which may be common clinical manifestations or co-occurrence hematological diseases of patients with SLE. All these findings suggest that IKZF1 may play a critical role in the pathogenesis of SLE. In this article, we discuss the existnig understanding of the role of IKZF1 on the physiological and pathological functions associated with SLE, providing insights that may assist in the development of new therapeutic strategies based on IKZF1 for patients with SLE.

Key words

IKZF1 - Pathogenesis - Systemic lupus erythematosus