The International Journal of Developmental Biology

Int. J. Dev. Biol. 63: 579 - 587 (2019)

Vol 63, Issue 11-12

Present status and expectation of aristaless-related homeobox (ARX) in endocrine pancreas

Review | Published: 27 February 2020

Sai Xu*,1,2 and Ji-Ping Xu*,1,2

1Shandong Provincial Key Laboratory of Animal Cells and Developmental Biology, School of Life Science, Shandong University, and 2Department of Neurology Medicine, Second Hospital of Shandong University, Jinan, Shandong, People's Republic of China


The aristaless-related homeobox (ARX) gene has become one of most frequently mutated genes which is closely linked with development of the vertebrate central nervous system; however, the molecular and clinical bases of its function in the proliferation and differentiation of the endocrine pancreas have not, to date, been systematically characterized. ARX is considered as a regulator which determines endocrine cell fate and a bio-marker of the pancreatic α-cell. Disruption and mutation of ARX are found to lead to the deletion and reduction of α-cells both in mice models and in humans. Furthermore, expression of ARX is regulated by multiple transcription factors involved in development of the pancreas, such as Ngn3, Isl1, Nkx2.2 and Nkx6.1. Taken together, given the vital importance of glucagon in diabetes treatment, it is possible that ARX may down-regulate exorbitant glucagon levels by reducing the number of α-cells as a direct target; thus, the role of ARX in the maintenance of α-cell identity and quantity should be investigated and summarized. This article mainly focuses on the role of ARX in the endocrine pancreas, introduces the ARX-related animal model and transcription factors, and highlights the latest advances in our understanding in order to provide a clearer theoretical foundation for future scientific research.


ARX, endocrine pancreas, transcription factor, mouse model, apoptosis

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