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Glycogen storage disease type Ib: role of glucose-6-phosphate transporter in cell metabolism and function

Authors
Sim, Sang WanWeinstein, David A.Lee, Young MokJun, Hyun Sik
Issue Date
1월-2020
Publisher
WILEY
Keywords
autoimmune disease; CD4(+) T cell; function; glucose-6-phosphate transporter; glycogen storage disease type Ib; macrophage; mesenchymal stem cell; metabolism; monocyte; neutrophil
Citation
FEBS LETTERS, v.594, no.1, pp.3 - 18
Indexed
SCIE
SCOPUS
Journal Title
FEBS LETTERS
Volume
594
Number
1
Start Page
3
End Page
18
URI
https://scholar.korea.ac.kr/handle/2021.sw.korea/58586
DOI
10.1002/1873-3468.13666
ISSN
0014-5793
Abstract
Cellular metabolism generally refers to biochemical processes that produce or consume energy within the cell. Recent studies have established that aberrant metabolic states caused by internal or external stresses and genetic mutations are intertwined with several human pathologies. Gaining insight into these metabolic alterations is, therefore, essential for understanding the pathophysiology of various diseases. Glycogen storage disease type Ib (GSD-Ib) is an autosomal recessive disorder characterized by hypoglycemia, excessive glycogen accumulation in the liver and kidney, neutropenia, neutrophil dysfunction, and inflammatory bowel disease. GSD-Ib is caused by a deficiency of glucose-6-phosphate transporter (G6PT). Recently, it was reported that deficiency of G6PT also leads to the aberrant proliferation and differentiation of mesenchymal stem cells and impaired regulatory T-cell function. This review describes the broad impact of altered cellular metabolism resulting from a lack of G6PT activity on cellular function and considers the prospects of developing novel approaches for GSD-Ib treatment.
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