日期:
2020 年 — 2026 年
2020
2021
2022
2023
2024
2025
2026
影响因子:

Intrinsic link between PGRN and Gba1 D409V mutation dosage in potentiating Gaucher disease

PGRN 和 Gba1 D409V 突变剂量之间的内在联系在增强戈谢病中的作用

Yi Lin ,Xiangli Zhao ,Benjamin Liou ,Venette Fannin ,Wujuan Zhang ,Kenneth D R Setchell ,Xiaohong Wang ,Dao Pan ,Gregory A Grabowski ,Chuan-Ju Liu ,Ying Sun

iPSC-derived neural precursor cells engineering GBA1 recovers acid β-glucosidase deficiency and diminishes α-synuclein and neuropathology

iPSC 衍生的神经前体细胞工程 GBA1 可恢复酸性 β-葡萄糖苷酶缺乏症并减少 α-突触核蛋白和神经病理学

Yanyan Peng, Benjamin Liou, Yi Lin, Christopher N Mayhew, Sheila M Fleming, Ying Sun

Analysis of the Biomarkers for Neurodegenerative Diseases in Aged Progranulin Deficient Mice

老年前颗粒蛋白缺乏小鼠神经退行性疾病生物标志物的分析

Xiangli Zhao, Sadaf Hasan, Benjamin Liou, Yi Lin, Ying Sun, Chuanju Liu

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

底物减少疗法可逆转戈谢病细胞模型中的线粒体、mTOR 和自噬改变

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

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

通过穿透血脑屏障的 SapC-DOPS 纳米囊泡进行全身酶输送,用于治疗神经性戈谢病

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

Intravenous infusion of iPSC-derived neural precursor cells increases acid β-glucosidase function in the brain and lessens the neuronopathic phenotype in a mouse model of Gaucher disease

静脉输注 iPSC 衍生的神经前体细胞可增强大脑中的酸性 β-葡萄糖苷酶功能,并减轻戈谢病小鼠模型中的神经病变表型

Yanyan Peng, Benjamin Liou, Venette Inskeep, Rachel Blackwood, Christopher N Mayhew, Gregory A Grabowski, Ying Sun

Complement drives glucosylceramide accumulation and tissue inflammation in Gaucher disease

补体驱动戈谢病中的葡萄糖神经酰胺积累和组织炎症

Manoj K Pandey, Thomas A Burrow, Reena Rani, Lisa J Martin, David Witte, Kenneth D Setchell, Mary A Mckay, Albert F Magnusen, Wujuan Zhang, Benjamin Liou, Jörg Köhl, Gregory A Grabowski

Neuronopathic Gaucher disease: dysregulated mRNAs and miRNAs in brain pathogenesis and effects of pharmacologic chaperone treatment in a mouse model

神经性戈谢病:小鼠模型中脑发病机制中失调的 mRNA 和 miRNA 以及药物伴侣治疗的影响

Nupur Dasgupta, You-Hai Xu, Ronghua Li, Yanyan Peng, Manoj K Pandey, Stuart L Tinch, Benjamin Liou, Venette Inskeep, Wujuan Zhang, Kenneth D R Setchell, Mehdi Keddache, Gregory A Grabowski, Ying Sun

Properties of neurons derived from induced pluripotent stem cells of Gaucher disease type 2 patient fibroblasts: potential role in neuropathology

戈谢病 2 型患者成纤维细胞诱导多能干细胞来源的神经元特性:在神经病理学中的潜在作用

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

The LIMP-2/SCARB2 binding motif on acid β-glucosidase: basic and applied implications for Gaucher disease and associated neurodegenerative diseases

酸性 β-葡萄糖苷酶上的 LIMP-2/SCARB2 结合基序:对戈谢病和相关神经退行性疾病的基础和应用意义

Benjamin Liou, Wendy D Haffey, Kenneth D Greis, Gregory A Grabowski