Comparative Therapeutic Effects of Velaglucerase Alfa and Imiglucerase in a Gaucher Disease Mouse Model

por Xu, You-Hai, Sun, Ying, Barnes, Sonya, Grabowski, Gregory A.
Publicado 2010

In Vivo and Ex Vivo Evaluation of L-Type Calcium Channel Blockers on Acid β-Glucosidase in Gaucher Disease Mouse Models

por Sun, Ying, Liou, Benjamin, Quinn, Brian, Ran, Huimin, Xu, You-Hai, Grabowski, Gregory A.
Publicado 2009

Gaucher Disease: Transcriptome Analyses Using Microarray or mRNA Sequencing in a Gba1 Mutant Mouse Model Treated with Velaglucerase alfa or Imiglucerase

por Dasgupta, Nupur, Xu, You-Hai, Oh, Sunghee, Sun, Ying, Jia, Li, Keddache, Mehdi, Grabowski, Gregory A
Publicado 2013

The role of UDP-Glc:glycoprotein glucosyltransferase 1 in the maturation of an obligate substrate prosaposin

por Pearse, Bradley R., Tamura, Taku, Sunryd, Johan C., Grabowski, Gregory A., Kaufman, Randal J., Hebert, Daniel N.
Publicado 2010

Progression of Behavioral and CNS Deficits in a Viable Murine Model of Chronic Neuronopathic Gaucher Disease

por Dai, Mei, Liou, Benjamin, Swope, Brittany, Wang, Xiaohong, Zhang, Wujuan, Inskeep, Venette, Grabowski, Gregory A., Sun, Ying, Pan, Dao
Publicado 2016

Substrate Compositional Variation with Tissue/Region and Gba1 Mutations in Mouse Models–Implications for Gaucher Disease

por Sun, Ying, Zhang, Wujuan, Xu, You-Hai, Quinn, Brian, Dasgupta, Nupur, Liou, Benjamin, Setchell, Kenneth D. R., Grabowski, Gregory A.
Publicado 2013

Ubiquitous Transgene Expression of the Glucosylceramide-Synthesizing Enzyme Accelerates Glucosylceramide Accumulation and Storage Cells in a Gaucher Disease Mouse Model

por Barnes, Sonya, Xu, You-Hai, Zhang, Wujuan, Liou, Benjamin, Setchell, Kenneth D. R., Bao, Liming, Grabowski, Gregory A., Sun, Ying
Publicado 2014

Systemic enzyme delivery by blood-brain barrier-penetrating SapC-DOPS nanovesicles for treatment of neuronopathic Gaucher disease

por Sun, Ying, Liou, Benjamin, Chu, Zhengtao, Fannin, Venette, Blackwood, Rachel, Peng, Yanyan, Grabowski, Gregory A., Davis, Harold W., Qi, Xiaoyang
Publicado 2020

Activation of p38 Mitogen-Activated Protein Kinase in Gaucher’s Disease

por Kitatani, Kazuyuki, Wada, Masayuki, Perry, David, Usui, Toshinori, Sun, Ying, Obeid, Lina M., Yaegashi, Nobuo, Grabowski, Gregory A., Hannun, Yusuf A.
Publicado 2015

Substrate Reduction Therapy Reverses Mitochondrial, mTOR, and Autophagy Alterations in a Cell Model of Gaucher Disease

por Peng, Yanyan, Liou, Benjamin, Lin, Yi, Fannin, Venette, Zhang, Wujuan, Feldman, Ricardo A., Setchell, Kenneth D. R., Grabowski, Gregory A., Sun, Ying
Publicado 2021

Global gene expression profile progression in Gaucher disease mouse models

por Xu, You-Hai, Jia, Li, Quinn, Brian, Zamzow, Matthew, Stringer, Keith, Aronow, Bruce, Sun, Ying, Zhang, Wujuan, Setchell, Kenneth DR, Grabowski, Gregory A
Publicado 2011

Combination of acid β-glucosidase mutation and Saposin C deficiency in mice reveals Gba1 mutation dependent and tissue-specific disease phenotype

por Liou, Benjamin, Zhang, Wujuan, Fannin, Venette, Quinn, Brian, Ran, Huimin, Xu, Kui, Setchell, Kenneth D. R., Witte, David, Grabowski, Gregory A., Sun, Ying
Publicado 2019

Temporal gene expression profiling reveals CEBPD as a candidate regulator of brain disease in prosaposin deficient mice

por Sun, Ying, Jia, Li, Williams, Michael T, Zamzow, Matt, Ran, Huimin, Quinn, Brian, Aronow, Bruce J, Vorhees, Charles V, Witte, David P, Grabowski, Gregory A
Publicado 2008

Specific saposin C deficiency: CNS impairment and acid β-glucosidase effects in the mouse

por Sun, Ying, Ran, Huimin, Zamzow, Matt, Kitatani, Kazuyuki, Skelton, Matthew R., Williams, Michael T., Vorhees, Charles V., Witte, David P., Hannun, Yusuf A., Grabowski, Gregory A.
Publicado 2010

Neurological deficits and glycosphingolipid accumulation in saposin B deficient mice

por Sun, Ying, Witte, David P., Ran, Huimin, Zamzow, Matt, Barnes, Sonya, Cheng, Hua, Han, Xianlin, Williams, Michael T., Skelton, Matthew R., Vorhees, Charles V., Grabowski, Gregory A.
Publicado 2008

Isofagomine In Vivo Effects in a Neuronopathic Gaucher Disease Mouse

por Sun, Ying, Ran, Huimin, Liou, Benjamin, Quinn, Brian, Zamzow, Matt, Zhang, Wujuan, Bielawski, Jacek, Kitatani, Kazuyuki, Setchell, Kenneth D. R., Hannun, Yusuf A., Grabowski, Gregory A.
Publicado 2011

Chitinase-3-like Protein 1: A Progranulin Downstream Molecule and Potential Biomarker for Gaucher Disease

por Jian, Jinlong, Chen, Yuehong, Liberti, Rossella, Fu, Wenyu, Hu, Wenhuo, Saunders-Pullman, Rachel, Pastores, Gregory M., Chen, Ying, Sun, Ying, Grabowski, Gregory A., Liu, Chuan-ju
Publicado 2018

PGRN deficiency exacerbates, whereas a brain penetrant PGRN derivative protects, GBA1 mutation-associated pathologies and diseases

por Zhao, Xiangli, Lin, Yi, Liou, Benjamin, Fu, Wenyu, Jian, Jinlong, Fannin, Venette, Zhang, Wujuan, Setchell, Kenneth D. R., Grabowski, Gregory A., Sun, Ying, Liu, Chuan-ju
Publicado 2022

Neuronopathic Gaucher disease in the mouse: viable combined selective saposin C deficiency and mutant glucocerebrosidase (V394L) mice with glucosylsphingosine and glucosylceramide...

por Sun, Ying, Liou, Benjamin, Ran, Huimin, Skelton, Matthew R., Williams, Michael T., Vorhees, Charles V., Kitatani, Kazuyuki, Hannun, Yusuf A., Witte, David P., Xu, You-Hai, Grabowski, Gregory A.
Publicado 2010

Properties of Neurons Derived from Induced Pluripotent Stem Cells of Gaucher Disease Type 2 Patient Fibroblasts: Potential Role in Neuropathology

por Sun, Ying, Florer, Jane, Mayhew, Christopher N., Jia, Zhanfeng, Zhao, Zhiying, Xu, Kui, Ran, Huimin, Liou, Benjamin, Zhang, Wujuan, Setchell, Kenneth D. R., Gu, Jianguo, Grabowski, Gregory A.
Publicado 2015