Aberrant proliferation and differentiation of glycogen storage disease type Ib mesenchymal stem cells.
- Sang Wan Sim,
- Tae Sub Park,
- Sung-Jo Kim,
- Byung-Chul Park,
- David A Weinstein,
- Young Mok Lee,
- Hyun Sik Jun
FEBS Lett 2018 Jan 3;592(2):162-171. Epub 2018 Jan 3.
Department of Biotechnology and Bioinformatics, College of Science and Technology, Korea University, Sejong, Korea.
Glycogen storage disease type Ib (GSD-Ib) is caused by mutations of the glucose-6-phosphate transporter (G6PT) and characterized by disrupted glucose homeostasis, neutropenia, and neutrophil dysfunction. To investigate the role of G6PT in human adipose-derived mesenchymal stem cells (hMSCs), the G6PT gene was mutated by CRISPR/Cas9 technology and single cell-derived G6PThMSCs were established. G6PThMSCs have significantly increased cell proliferation but impaired adipogenesis and osteogenesis. Read More