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Aberrant glucose metabolism underlies impaired macrophage differentiation in glycogen storage disease type Ib.
Jang, Yuyeon; Park, Tae Sub; Park, Byung-Chul; Lee, Young Mok; Heo, Tae-Hwe; Jun, Hyun Sik FASEB journal : official publication of the Federation of American Societies for Experimental Biology 2023 Nov;37(11):e23216
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A Mouse Model of Glycogen Storage Disease Type IX-Beta: A Role for Phkb in Glycogenolysis.
Arends, Charles J; Wilson, Lane H; Estrella, Ana; Kwon, Oh Sung; Weinstein, David A; Lee, Young Mok International journal of molecular sciences 2022 Sep;23(17):
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Molecular mechanisms of aberrant neutrophil differentiation in glycogen storage disease type Ib.
Sim, Sang Wan; Jang, Yuyeon; Park, Tae Sub; Park, Byung-Chul; Lee, Young Mok; Jun, Hyun Sik Cellular and molecular life sciences : CMLS 2022 Apr;79(5):246
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Exosomal MicroRNAs as Potential Biomarkers of Hepatic Injury and Kidney Disease in Glycogen Storage Disease Type Ia Patients.
Resaz, Roberta; Cangelosi, Davide; Segalerba, Daniela; Morini, Martina; Uva, Paolo; Bosco, Maria Carla; Banderali, Giuseppe; Estrella, Ana; Wanner, Corbinian; Weinstein, David A; Sechi, Annalisa; Paci, Sabrina; Melis, Daniela; Di Rocco, Maja; Lee, Young Mok; Eva, Alessandra International journal of molecular sciences 2021 Dec;23(1):
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Emerging roles of autophagy in hepatic tumorigenesis and therapeutic strategies in glycogen storage disease type Ia: a review.
Cho, Jun-Ho; Weinstein, David A; Lee, Young Mok Journal of inherited metabolic disease 2020 May;
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Glucose-6-phosphate transporter mediates macrophage proliferation and functions by regulating glycolysis and mitochondrial respiration.
Jeon, Eek Hyung; Park, Tae Sub; Jang, Yuyeon; Hwang, Eunmi; Kim, Sung-Jo; Song, Ki-Duk; Weinstein, David A; Lee, Young Mok; Park, Byung-Chul; Jun, Hyun Sik Biochemical and biophysical research communications 2020 Jan;
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Activation of tumor-promoting pathways implicated in hepatocellular adenoma/carcinoma, a long-term complication of glycogen storage disease type Ia.
Cho, Jun-Ho; Lee, Young Mok; Bae, Seong-Ho; Chou, Janice Y Biochemical and biophysical research communications 2019 Nov;
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Glycogen storage disease type Ib: role of glucose-6-phosphate transporter in cell metabolism and function.
Sim, Sang Wan; Weinstein, David A; Lee, Young Mok; Jun, Hyun Sik FEBS letters 2019 Nov;
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Liver Glycogen Phosphorylase Deficiency Leads to Profibrogenic Phenotype in a Murine Model of Glycogen Storage Disease Type VI.
Wilson, Lane H; Cho, Jun-Ho; Estrella, Ana; Smyth, Joan A; Wu, Rong; Chengsupanimit, Tayoot; Brown, Laurie M; Weinstein, David A; Lee, Young Mok Hepatology communications 2019 Nov;3(11):1544-1555
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Gene therapy prevents hepatic tumor initiation in murine glycogen storage disease type Ia at the tumor-developing stage.
Cho, Jun-Ho; Lee, Young Mok; Starost, Matthew F; Mansfield, Brian C; Chou, Janice Y Journal of inherited metabolic disease 2019 May;42(3):459-469
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Long-term safety and efficacy of AAV gene therapy in the canine model of glycogen storage disease type Ia.
Lee, Young Mok; Conlon, Thomas J; Specht, Andrew; Coleman, Kirsten E; Brown, Laurie M; Estrella, Ana M; Dambska, Monika; Dahlberg, Kathryn R; Weinstein, David A Journal of inherited metabolic disease 2018 May;41977-984
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Aberrant proliferation and differentiation of glycogen storage disease type Ib mesenchymal stem cells.
Sim, Sang Wan; Park, Tae Sub; Kim, Sung-Jo; Park, Byung-Chul; Weinstein, David A; Lee, Young Mok; Jun, Hyun Sik FEBS letters 2017 Dec;592162-171
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Liver-directed gene therapy for murine glycogen storage disease type Ib.
Kwon, Joon Hyun; Lee, Young Mok; Cho, Jun-Ho; Kim, Goo-Young; Anduaga, Javier; Starost, Matthew F; Mansfield, Brian C; Chou, Janice Y Human molecular genetics 2017 Nov;26(22):4395-4405
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Glycogen storage disease type Ia mice with less than 2% of normal hepatic glucose-6-phosphatase-α activity restored are at risk of developing hepatic tumors.
Kim, Goo-Young; Lee, Young Mok; Kwon, Joon Hyun; Cho, Jun-Ho; Pan, Chi-Jiunn; Starost, Matthew F; Mansfield, Brian C; Chou, Janice Y Molecular genetics and metabolism 2017 Mar;120(3):229-234
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Glycogen storage disease type Ib neutrophils exhibit impaired cell adhesion and migration.
Kim, Goo-Young; Lee, Young Mok; Kwon, Joon Hyun; Jun, Hyun Sik; Chou, Janice Biochemical and biophysical research communications 2017 Jan;482(4):569-574
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Mice expressing reduced levels of hepatic glucose-6-phosphatase-α activity do not develop age-related insulin resistance or obesity.
Kim, Goo-Young; Lee, Young Mok; Cho, Jun-Ho; Pan, Chi-Jiunn; Jun, Hyun Sik; Springer, Danielle A; Mansfield, Brian C; Chou, Janice Y Human molecular genetics 2015 Sep;24(18):5115-25
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Minimal hepatic glucose-6-phosphatase-α activity required to sustain survival and prevent hepatocellular adenoma formation in murine glycogen storage disease type Ia.
Lee, Young Mok; Kim, Goo-Young; Pan, Chi-Jiunn; Mansfield, Brian C; Chou, Janice Y Molecular genetics and metabolism reports 2015 Jun;328-32
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Molecular mechanisms of neutrophil dysfunction in glycogen storage disease type Ib.
Jun, Hyun Sik; Weinstein, David A; Lee, Young Mok; Mansfield, Brian C; Chou, Janice Y Blood 2014 May;123(18):2843-53
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The upstream enhancer elements of the G6PC promoter are critical for optimal G6PC expression in murine glycogen storage disease type Ia.
Lee, Young Mok; Pan, Chi-Jiunn; Koeberl, Dwight D; Mansfield, Brian C; Chou, Janice Y Molecular genetics and metabolism 2013 Nov;110(3):275-80
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Prevention of hepatocellular adenoma and correction of metabolic abnormalities in murine glycogen storage disease type Ia by gene therapy.
Lee, Young Mok; Jun, Hyun Sik; Pan, Chi-Jiunn; Lin, Su Ru; Wilson, Lane H; Mansfield, Brian C; Chou, Janice Y Hepatology (Baltimore, Md.) 2012 Nov;56(5):1719-29
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Glucose-6-phosphatase-β, implicated in a congenital neutropenia syndrome, is essential for macrophage energy homeostasis and functionality.
Jun, Hyun Sik; Cheung, Yuk Yin; Lee, Young Mok; Mansfield, Brian C; Chou, Janice Y Blood 2012 Apr;119(17):4047-55
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G-CSF improves murine G6PC3-deficient neutrophil function by modulating apoptosis and energy homeostasis.
Jun, Hyun Sik; Lee, Young Mok; Song, Ki Duk; Mansfield, Brian C; Chou, Janice Y Blood 2011 Apr;117(14):3881-92
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Glycogen storage disease type Ia in canines: a model for human metabolic and genetic liver disease.
Specht, Andrew; Fiske, Laurie; Erger, Kirsten; Cossette, Travis; Verstegen, John; Campbell-Thompson, Martha; Struck, Maggie B; Lee, Young Mok; Chou, Janice Y; Byrne, Barry J; Correia, Catherine E; Mah, Cathryn S; Weinstein, David A; Conlon, Thomas J Journal of biomedicine & biotechnology 2011 Jan;2011646257
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Gamma-irradiation depletes endogenous germ cells and increases donor cell distribution in chimeric chickens.
Park, Kyung Je; Kang, Seok Jin; Kim, Tae Min; Lee, Young Mok; Lee, Hyung Chul; Song, Gwonhwa; Han, Jae Yong In vitro cellular & developmental biology. Animal 2010 Dec;46(10):828-33
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Lack of glucose recycling between endoplasmic reticulum and cytoplasm underlies cellular dysfunction in glucose-6-phosphatase-beta-deficient neutrophils in a congenital neutropenia syndrome.
Jun, Hyun Sik; Lee, Young Mok; Cheung, Yuk Yin; McDermott, David H; Murphy, Philip M; De Ravin, Suk See; Mansfield, Brian C; Chou, Janice Y Blood 2010 Oct;116(15):2783-92
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Complete normalization of hepatic G6PC deficiency in murine glycogen storage disease type Ia using gene therapy.
Yiu, Wai Han; Lee, Young Mok; Peng, Wen-Tao; Pan, Chi-Jiunn; Mead, Paul A; Mansfield, Brian C; Chou, Janice Y Molecular therapy : the journal of the American Society of Gene Therapy 2010 Jun;18(6):1076-84
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The reversible developmental unipotency of germ cells in chicken.
Jung, Jin Gyoung; Lee, Young Mok; Kim, Jin Nam; Kim, Tae Min; Shin, Ji Hye; Kim, Tae Hyun; Lim, Jeong Mook; Han, Jae Yong Reproduction (Cambridge, England) 2010 Jan;139(1):113-9
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Development of a pheasant interspecies primordial germ cell transfer to chicken embryo: effect of donor cell sex on chimeric semen production.
Kang, S J; Choi, J W; Park, K J; Lee, Y M; Kim, T M; Sohn, S H; Lim, J M; Han, J Y Theriogenology 2009 Sep;72(4):519-27
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Reproduction of wild birds via interspecies germ cell transplantation.
Kang, Seok Jin; Choi, Jin Won; Kim, Sun Young; Park, Kyung Je; Kim, Tae Min; Lee, Young Mok; Kim, Heebal; Lim, Jeong Mook; Han, Jae Yong Biology of reproduction 2008 Nov;79(5):931-7
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Identification, culture, and characterization of germline stem cell-like cells in chicken testes.
Jung, Jin Gyoung; Lee, Young Mok; Park, Tae Sub; Park, Sang Hyun; Lim, Jeong Mook; Han, Jae Yong Biology of reproduction 2007 Jan;76(1):173-82
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A testis-mediated germline chimera production based on transfer of chicken testicular cells directly into heterologous testes.
Lee, Young Mok; Jung, Jin Gyoung; Kim, Jin Nam; Park, Tae Sub; Kim, Tae Min; Shin, Sang Su; Kang, Dae Kyung; Lim, Jeong Mook; Han, Jae Yong Biology of reproduction 2006 Sep;75(3):380-6
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Detection and characterization of primordial germ cells in pheasant (Phasianus colchicus) embryos.
Kim, Jin Nam; Lee, Young Mok; Park, Tae Sub; Jung, Jin Gyoung; Cho, Byeong Wook; Lim, Jeong Mook; Han, Jae Yong Theriogenology 2005 Mar;63(4):1038-49