Molecules and Cells

Korean Society for Molecular and Cellular Biology (한국분자세포생물학회)
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Cell Death and Stress Signaling in Glycogen Storage Disease Type I

  • Kim, So Youn (Center for Cell Signaling and Drug Discovery Research (National Core Research Center), Ewha Womans University) ;
  • Bae, Yun Soo (Center for Cell Signaling and Drug Discovery Research (National Core Research Center), Ewha Womans University)
  • Received : 2009.08.17
  • Accepted : 2009.08.19
  • Published : 2009.09.30
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Abstract

Cell death has been traditionally classified in apoptosis and necrosis. Apoptosis, known as programmed cell death, is an active form of cell death mechanism that is tightly regulated by multiple cellular signaling pathways and requires ATP for its appropriate process. Apoptotic death plays essential roles for successful development and maintenance of normal cellular homeostasis in mammalian. In contrast to apoptosis, necrosis is classically considered as a passive cell death process that occurs rather by accident in disastrous conditions, is not required for energy and eventually induces inflammation. Regardless of different characteristics between apoptosis and necrosis, it has been well defined that both are responsible for a wide range of human diseases. Glycogen storage disease type I (GSD-I) is a kind of human genetic disorders and is caused by the deficiency of a microsomal protein, glucose-6-phosphatase-${\alpha}$ ($G6Pase-{\alpha}$) or glucose-6-phosphate transporter (G6PT) responsible for glucose homeostasis, leading to GSD-Ia or GSD-Ib, respectively. This review summarizes cell deaths in GSD-I and mostly focuses on current knowledge of the neutrophil apoptosis in GSD-Ib based upon ER stress and redox signaling.

Keywords

Acknowledgement

Supported by : Korea Research Foundation, Korea Science and Engineering Foundation, Ewha Womans University

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