Hasil Pencarian - Gregory A Grabowski

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  1. 1
    Gaucher disease mouse models: point mutations at the acid β-glucosidase locus combined with low-level prosaposin expression lead to disease variants

    Gaucher disease mouse models: point mutations at the acid β-glucosidase locus combined with low-level prosaposin expression lead to disease variants oleh Ying Sun, Brian Quinn, David P. Witte, Gregory A. Grabowski

    Diterbitkan 2005-10-01
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  2. 2
    Multi-system disorders of glycosphingolipid and ganglioside metabolism

    Multi-system disorders of glycosphingolipid and ganglioside metabolism oleh You-Hai Xu, Sonya Barnes, Ying Sun, Gregory A. Grabowski

    Diterbitkan 2010-07-01
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  3. 3
    Comparative therapeutic effects of velaglucerase alfa and imiglucerase in a Gaucher disease mouse model.

    Comparative therapeutic effects of velaglucerase alfa and imiglucerase in a Gaucher disease mouse model. oleh You-Hai Xu, Ying Sun, Sonya Barnes, Gregory A Grabowski

    Diterbitkan 2010-05-01
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  4. 4
    In vivo and ex vivo evaluation of L-type calcium channel blockers on acid beta-glucosidase in Gaucher disease mouse models.

    In vivo and ex vivo evaluation of L-type calcium channel blockers on acid beta-glucosidase in Gaucher disease mouse models. oleh Ying Sun, Benjamin Liou, Brian Quinn, Huimin Ran, You-Hai Xu, Gregory A Grabowski

    Diterbitkan 2009-10-01
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  5. 5
    Conditional expression of human acid β-glucosidase improves the visceral phenotype in a Gaucher disease mouse model

    Conditional expression of human acid β-glucosidase improves the visceral phenotype in a Gaucher disease mouse model oleh Ying Sun, Brian Quinn, You-Hai Xu, Tatyana Leonova, David P. Witte, Gregory A. Grabowski

    Diterbitkan 2006-10-01
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  6. 6
    Gaucher disease: transcriptome analyses using microarray or mRNA sequencing in a Gba1 mutant mouse model treated with velaglucerase alfa or imiglucerase.

    Gaucher disease: transcriptome analyses using microarray or mRNA sequencing in a Gba1 mutant mouse model treated with velaglucerase alfa or imiglucerase. oleh Nupur Dasgupta, You-Hai Xu, Sunghee Oh, Ying Sun, Li Jia, Mehdi Keddache, Gregory A Grabowski

    Diterbitkan 2013-01-01
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  7. 7
    Lysosomal acid lipase-deficient mice: depletion of white and brown fat, severe hepatosplenomegaly, and shortened life span

    Lysosomal acid lipase-deficient mice: depletion of white and brown fat, severe hepatosplenomegaly, and shortened life span oleh Hong Du, Martin Heur, Ming Duanmu, Gregory A. Grabowski, David Y. Hui, David P. Witte, Jaya Mishra

    Diterbitkan 2001-04-01
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  8. 8
    Progression of Behavioral and CNS Deficits in a Viable Murine Model of Chronic Neuronopathic Gaucher Disease.

    Progression of Behavioral and CNS Deficits in a Viable Murine Model of Chronic Neuronopathic Gaucher Disease. oleh Mei Dai, Benjamin Liou, Brittany Swope, Xiaohong Wang, Wujuan Zhang, Venette Inskeep, Gregory A Grabowski, Ying Sun, Dao Pan

    Diterbitkan 2016-01-01
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  9. 9
    Substrate compositional variation with tissue/region and Gba1 mutations in mouse models--implications for Gaucher disease.

    Substrate compositional variation with tissue/region and Gba1 mutations in mouse models--implications for Gaucher disease. oleh Ying Sun, Wujuan Zhang, You-Hai Xu, Brian Quinn, Nupur Dasgupta, Benjamin Liou, Kenneth D R Setchell, Gregory A Grabowski

    Diterbitkan 2013-01-01
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  10. 10
    Ubiquitous transgene expression of the glucosylceramide-synthesizing enzyme accelerates glucosylceramide accumulation and storage cells in a Gaucher disease mouse model.

    Ubiquitous transgene expression of the glucosylceramide-synthesizing enzyme accelerates glucosylceramide accumulation and storage cells in a Gaucher disease mouse model. oleh Sonya Barnes, You-Hai Xu, Wujuan Zhang, Benjamin Liou, Kenneth D R Setchell, Liming Bao, Gregory A Grabowski, Ying Sun

    Diterbitkan 2014-01-01
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  11. 11
    Systemic enzyme delivery by blood-brain barrier-penetrating SapC-DOPS nanovesicles for treatment of neuronopathic Gaucher disease

    Systemic enzyme delivery by blood-brain barrier-penetrating SapC-DOPS nanovesicles for treatment of neuronopathic Gaucher disease oleh Ying Sun, Benjamin Liou, Zhengtao Chu, Venette Fannin, Rachel Blackwood, Yanyan Peng, Gregory A. Grabowski, Harold W. Davis, Xiaoyang Qi

    Diterbitkan 2020-05-01
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  12. 12
    Apolipoprotein E-deficient lipoproteins induce foam cell formation by downregulation of lysosomal hydrolases in macrophages

    Apolipoprotein E-deficient lipoproteins induce foam cell formation by downregulation of lysosomal hydrolases in macrophages oleh DongFang Wu, Chakradhari Sharan, Hong Yang, J. Shawn Goodwin, Lichun Zhou, Gregory A. Grabowski, Hong Du, ZhongMao Guo

    Diterbitkan 2007-12-01
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  13. 13
    Activation of p38 Mitogen-Activated Protein Kinase in Gaucher's Disease.

    Activation of p38 Mitogen-Activated Protein Kinase in Gaucher's Disease. oleh Kazuyuki Kitatani, Masayuki Wada, David Perry, Toshinori Usui, Ying Sun, Lina M Obeid, Nobuo Yaegashi, Gregory A Grabowski, Yusuf A Hannun

    Diterbitkan 2015-01-01
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  14. 14
    Substrate Reduction Therapy Reverses Mitochondrial, mTOR, and Autophagy Alterations in a Cell Model of Gaucher Disease

    Substrate Reduction Therapy Reverses Mitochondrial, mTOR, and Autophagy Alterations in a Cell Model of Gaucher Disease oleh Yanyan Peng, Benjamin Liou, Yi Lin, Venette Fannin, Wujuan Zhang, Ricardo A. Feldman, Kenneth D. R. Setchell, Gregory A. Grabowski, Ying Sun

    Diterbitkan 2021-09-01
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  15. 15
    Wolman disease/cholesteryl ester storage disease: efficacy of plant-produced human lysosomal acid lipase in mice*

    Wolman disease/cholesteryl ester storage disease: efficacy of plant-produced human lysosomal acid lipase in mice* oleh Hong Du, Terri L. Cameron, Stephen J. Garger, Gregory P. Pogue, Lee A. Hamm, Earl White, Kathleen M. Hanley, Gregory A. Grabowski

    Diterbitkan 2008-08-01
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  16. 16
    Endogenous β-glucocerebrosidase activity in Abca12−/−epidermis elevates ceramide levels after topical lipid application but does not restore barrier function

    Endogenous β-glucocerebrosidase activity in Abca12−/−epidermis elevates ceramide levels after topical lipid application but does not restore barrier function oleh Jorge F. Haller, Paul Cavallaro, Nicholas J. Hernandez, Lee Dolat, Stephanie J. Soscia, Ruth Welti, Gregory A. Grabowski, Michael L. Fitzgerald, Mason W. Freeman

    Diterbitkan 2014-03-01
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  17. 17
    Isofagomine in vivo effects in a neuronopathic Gaucher disease mouse.

    Isofagomine in vivo effects in a neuronopathic Gaucher disease mouse. oleh Ying Sun, Huimin Ran, Benjamin Liou, Brian Quinn, Matt Zamzow, Wujuan Zhang, Jacek Bielawski, Kazuyuki Kitatani, Kenneth D R Setchell, Yusuf A Hannun, Gregory A Grabowski

    Diterbitkan 2011-04-01
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  18. 18
    Chitinase-3-like Protein 1: A Progranulin Downstream Molecule and Potential Biomarker for Gaucher Disease

    Chitinase-3-like Protein 1: A Progranulin Downstream Molecule and Potential Biomarker for Gaucher Disease oleh Jinlong Jian, Yuehong Chen, Rossella Liberti, Wenyu Fu, Wenhuo Hu, Rachel Saunders-Pullman, Gregory M. Pastores, Ying Chen, Ying Sun, Gregory A. Grabowski, Chuan-ju Liu

    Diterbitkan 2018-02-01
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  19. 19
    Properties of neurons derived from induced pluripotent stem cells of Gaucher disease type 2 patient fibroblasts: potential role in neuropathology.

    Properties of neurons derived from induced pluripotent stem cells of Gaucher disease type 2 patient fibroblasts: potential role in neuropathology. oleh Ying Sun, Jane Florer, Christopher N Mayhew, Zhanfeng Jia, Zhiying Zhao, Kui Xu, Huimin Ran, Benjamin Liou, Wujuan Zhang, Kenneth D R Setchell, Jianguo Gu, Gregory A Grabowski

    Diterbitkan 2015-01-01
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  20. 20
    Association Between Progranulin and Gaucher Disease

    Association Between Progranulin and Gaucher Disease oleh Jinlong Jian, Shuai Zhao, Qing-Yun Tian, Helen Liu, Yunpeng Zhao, Wen-Chi Chen, Gabriele Grunig, Paola A. Torres, Betty C. Wang, Bai Zeng, Gregory Pastores, Wei Tang, Ying Sun, Gregory A. Grabowski, Max Xiangtian Kong, Guilin Wang, Ying Chen, Fengxia Liang, Herman S. Overkleeft, Rachel Saunders-Pullman, Gerald L. Chan, Chuan-ju Liu

    Diterbitkan 2016-09-01
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