Cell death and stress signaling in glycogen storage disease type I

So Youn Kim, Yun Soo Bae

Research output: Contribution to journalShort surveypeer-review

5 Scopus citations


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-α (G6Pase-α) 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.

Original languageEnglish
Pages (from-to)139-148
Number of pages10
JournalMolecules and Cells
Issue number3
StatePublished - 2009

Bibliographical note

Funding Information:
This work was supported b y the Korea Research Foundation Grant funded by the Korean Government (Ministry of Education and Human Resources Development, Basic Research Promotion Fund) (KRF-2008-359-C00030) and b y the National Core Research Center program (grant R15-2006-020-00000-0) of the Ministry of Education, Science and Technology/Korea Science and Engineering Foundation through the Center for Cell Signaling and Drug Discovery Research at Ewha Womans University.


  • Apoptosis
  • Autophagy
  • ER stress
  • GSD-I
  • Immune response
  • NADPH oxidase
  • Necrosis
  • Oxidative stress
  • Redox signaling
  • Toll-like receptor


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