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

Disruption of lysosomal nutrient sensing scaffold contributes to pathogenesis of a fatal neurodegenerative lysosomal storage disease

溶酶体营养传感支架的破坏导致致命的神经退行性溶酶体贮积症的发病机制

Maria B Bagh, Abhilash P Appu, Tamal Sadhukhan, Avisek Mondal, Nisha Plavelil, Mahadevan Raghavankutty, Ajayan M Supran, Sriparna Sadhukhan, Aiyi Liu, Anil B Mukherjee

Ppt1-deficiency dysregulates lysosomal Ca++ homeostasis contributing to pathogenesis in a mouse model of CLN1 disease

Ppt1 缺乏导致溶酶体 Ca++ 稳态失调,导致 CLN1 疾病小鼠模型发病

Avisek Mondal, Abhilash P Appu, Tamal Sadhukhan, Maria B Bagh, Rafael M Previde, Sriparna Sadhukhan, Stanko Stojilkovic, Aiyi Liu, Anil B Mukherjee

Cln1-mutations suppress Rab7-RILP interaction and impair autophagy contributing to neuropathology in a mouse model of infantile neuronal ceroid lipofuscinosis

Cln1 突变可抑制 Rab7-RILP 相互作用并损害自噬,导致婴儿神经元蜡样脂褐素沉积症小鼠模型中的神经病理学

Chinmoy Sarkar, Tamal Sadhukhan, Maria B Bagh, Abhilash P Appu, Goutam Chandra, Avisek Mondal, Arjun Saha, Anil B Mukherjee

Cln3-mutations underlying juvenile neuronal ceroid lipofuscinosis cause significantly reduced levels of Palmitoyl-protein thioesterases-1 (Ppt1)-protein and Ppt1-enzyme activity in the lysosome

青少年神经元蜡样脂褐素沉积症的 Cln3 突变导致溶酶体中棕榈酰蛋白硫酯酶-1 (Ppt1) 蛋白和 Ppt1 酶活性显著降低

Abhilash P Appu, Maria B Bagh, Tamal Sadhukhan, Avisek Mondal, Sydney Casey, Anil B Mukherjee

Misrouting of v-ATPase subunit V0a1 dysregulates lysosomal acidification in a neurodegenerative lysosomal storage disease model

v-ATPase 亚基 V0a1 的错误路由导致神经退行性溶酶体贮积症模型中的溶酶体酸化失调

Maria B Bagh, Shiyong Peng, Goutam Chandra, Zhongjian Zhang, Satya P Singh, Nagarajan Pattabiraman, Aiyi Liu, Anil B Mukherjee

Suppression of agrin-22 production and synaptic dysfunction in Cln1 (-/-) mice

抑制 Cln1 (-/-) 小鼠中的 agrin-22 产生和突触功能障碍

Shiyong Peng, Jianhua Xu, Kenneth A Pelkey, Goutam Chandra, Zhongjian Zhang, Maria B Bagh, Xiaoqing Yuan, Ling-Gang Wu, Chris J McBain, Anil B Mukherjee

Cln1 gene disruption in mice reveals a common pathogenic link between two of the most lethal childhood neurodegenerative lysosomal storage disorders

小鼠的 Cln1 基因破坏揭示了两种最致命的儿童神经退行性溶酶体贮积症之间的共同致病联系

Goutam Chandra, Maria B Bagh, Shiyong Peng, Arjun Saha, Chinmoy Sarkar, Matthew Moralle, Zhongjian Zhang, Anil B Mukherjee