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Items: 1 to 20 of 61

1.
Disruption of lysosomal nutrient sensing scaffold contributes to pathogenesis of a fatal neurodegenerative lysosomal storage disease
Maria B. Bagh, Abhilash P. Appu, Tamal Sadhukhan, Avisek Mondal, Nisha Plavelil, Mahadevan Raghavankutty, Ajayan M. Supran, Sriparna Sadhukhan, Aiyi Liu, Anil B. Mukherjee
J Biol Chem. 2024 Feb; 300(2): 105641. Published online 2024 Jan 9. doi: 10.1016/j.jbc.2024.105641
PMCID:
PMC10862020
AbstractArticlePDF–4.5M
2.
Cyclical palmitoylation regulates TLR9 signalling and systemic autoimmunity in mice
Hai Ni, Yinuo Wang, Kai Yao, Ling Wang, Jiancheng Huang, Yongfang Xiao, Hongyao Chen, Bo Liu, Cliff Y. Yang, Jijun Zhao
Nat Commun. 2024; 15: 1. Published online 2024 Jan 2. doi: 10.1038/s41467-023-43650-z
PMCID:
PMC10762000
AbstractArticlePDF–2.7M
3.
An innovative hematopoietic stem cell gene therapy approach benefits CLN1 disease in the mouse model
Marco Peviani, Sabyasachi Das, Janki Patel, Odella Jno‐Charles, Rajesh Kumar, Ana Zguro, Tyler D Mathews, Paolo Cabras, Rita Milazzo, Eleonora Cavalca, Valentina Poletti, Alessandra Biffi
EMBO Mol Med. 2023 Apr; 15(4): e15968. Published online 2023 Mar 6. doi: 10.15252/emmm.202215968
PMCID:
PMC10086581
AbstractArticlePDF–3.5M
4.
Effects of chronic cannabidiol in a mouse model of naturally occurring neuroinflammation, neurodegeneration, and spontaneous seizures
Joshua T. Dearborn, Hemanth R. Nelvagal, Nicholas R. Rensing, Keigo Takahashi, Stephanie M. Hughes, Thomas M. Wishart, Jonathan D. Cooper, Michael Wong, Mark S. Sands
Sci Rep. 2022; 12: 11286. Published online 2022 Jul 4. doi: 10.1038/s41598-022-15134-5
PMCID:
PMC9253004
AbstractArticlePDF–4.3M
5.
Seizures in PPT1 Knock-In Mice Are Associated with Inflammatory Activation of Microglia
Xusheng Zhang, Mengting Wang, Bingyan Feng, Qiuyu Zhang, Jia Tong, Mingyong Wang, Chengbiao Lu, Shiyong Peng
Int J Mol Sci. 2022 May; 23(10): 5586. Published online 2022 May 17. doi: 10.3390/ijms23105586
PMCID:
PMC9144763
AbstractArticlePDF–4.9M
6.
Ppt1-deficiency dysregulates lysosomal Ca++-homeostasis contributing to pathogenesis in a mouse model of CLN1 disease
Avisek Mondal, Abhilash P. Appu, Tamal Sadhukhan, Maria B. Bagh, Rafael M. Previde, Sriparna Sadhukhan, Stanko Stojilkovic, Aiyi Liu, Anil B Mukherjee
J Inherit Metab Dis. Author manuscript; available in PMC 2023 May 1.
Published in final edited form as: J Inherit Metab Dis. 2022 May; 45(3): 635–656. Published online 2022 Mar 17. doi: 10.1002/jimd.12485
PMCID:
PMC9090967
AbstractArticlePDF–3.2M
7.
Identification of substrates of palmitoyl protein thioesterase 1 highlights roles of depalmitoylation in disulfide bond formation and synaptic function
Erica L. Gorenberg, Sofia Massaro Tieze, Betül Yücel, Helen R. Zhao, Vicky Chou, Gregory S. Wirak, Susumu Tomita, TuKiet T. Lam, Sreeganga S. Chandra
PLoS Biol. 2022 Mar; 20(3): e3001590. Published online 2022 Mar 31. doi: 10.1371/journal.pbio.3001590
PMCID:
PMC9004782
AbstractArticlePDF–3.2M
8.
Cln1-mutations suppress Rab7-RILP interaction and impair autophagy contributing to neuropathology in a mouse model of INCL
Chinmoy Sarkar, Tamal Sadhukhan, Maria B. Bagh, Abhilash P. Appu, Goutam Chandra, Avisek Mondal, Arjun Saha, Anil B. Mukherjee
J Inherit Metab Dis. Author manuscript; available in PMC 2021 Sep 1.
Published in final edited form as: J Inherit Metab Dis. 2020 Sep; 43(5): 1082–1101. Published online 2020 Apr 27. doi: 10.1002/jimd.12242
PMCID:
PMC8261861
AbstractArticlePDF–2.6M
9.
GFAP hyperpalmitoylation exacerbates astrogliosis and neurodegenerative pathology in PPT1-deficient mice
Wei Yuan, Liaoxun Lu, Muding Rao, Yang Huang, Chun-e Liu, Shuang Liu, Yue Zhao, Huicong Liu, Jiangli Zhu, Tianzhu Chao, Can Wu, Junyan Ren, Luxian Lv, Wenqiang Li, Shiqian Qi, Yinming Liang, Shijing Yue, Jian Gao, Zhongjian Zhang, Eryan Kong
Proc Natl Acad Sci U S A. 2021 Mar 30; 118(13): e2022261118. Published online 2021 Mar 22. doi: 10.1073/pnas.2022261118
PMCID:
PMC8020761
AbstractArticlePDF–2.2M
10.
Fronto-temporal dementia risk gene TMEM106B has opposing effects in different lysosomal storage disorders
Azucena Perez-Canamas, Hideyuki Takahashi, Jane A Lindborg, Stephen M Strittmatter
Brain Commun. 2021; 3(1): fcaa200. Published online 2020 Nov 16. doi: 10.1093/braincomms/fcaa200
PMCID:
PMC7990118
AbstractArticlePDF–1.7M
11.
Sex- and region-biased depletion of microglia/macrophages attenuates CLN1 disease in mice
Kristina Berve, Brian L. West, Rudolf Martini, Janos Groh
J Neuroinflammation. 2020; 17: 323. Published online 2020 Oct 28. doi: 10.1186/s12974-020-01996-x
PMCID:
PMC7594417
AbstractArticlePDF–5.1M
12.
Comparative proteomic profiling reveals mechanisms for early spinal cord vulnerability in CLN1 disease
Hemanth R. Nelvagal, Maica Llavero Hurtado, Samantha L. Eaton, Rachel A. Kline, Douglas J. Lamont, Mark S. Sands, Thomas M. Wishart, Jonathan D. Cooper
Sci Rep. 2020; 10: 15157. Published online 2020 Sep 16. doi: 10.1038/s41598-020-72075-7
PMCID:
PMC7495486
AbstractArticlePDF–5.9M
13.
Ex vivo Quantitative Proteomic Analysis of Serotonin Transporter Interactome: Network Impact of the SERT Ala56 Coding Variant
Meagan A. Quinlan, Matthew J. Robson, Ran Ye, Kristie L. Rose, Kevin L. Schey, Randy D. Blakely
Front Mol Neurosci. 2020; 13: 89. Published online 2020 Jun 8. doi: 10.3389/fnmol.2020.00089
PMCID:
PMC7295033
AbstractArticlePDF–2.7M
14.
Mouse Models of Inherited Retinal Degeneration with Photoreceptor Cell Loss
Gayle B. Collin, Navdeep Gogna, Bo Chang, Nattaya Damkham, Jai Pinkney, Lillian F. Hyde, Lisa Stone, Jürgen K. Naggert, Patsy M. Nishina, Mark P. Krebs
Cells. 2020 Apr; 9(4): 931. Published online 2020 Apr 10. doi: 10.3390/cells9040931
PMCID:
PMC7227028
AbstractArticlePDF–5.4M
15.
Mice deficient in the lysosomal enzyme palmitoyl-protein thioesterase 1 (PPT1) display a complex retinal phenotype
Yevgeniya Atiskova, Susanne Bartsch, Tatyana Danyukova, Elke Becker, Christian Hagel, Stephan Storch, Udo Bartsch
Sci Rep. 2019; 9: 14185. Published online 2019 Oct 2. doi: 10.1038/s41598-019-50726-8
PMCID:
PMC6775149
AbstractArticlePDF–3.2M
16.
Congenic Mapping of Alcohol and Pentobarbital Withdrawal Liability Loci to a <1 Centimorgan Interval of Murine Chromosome 4: Identification of Mpdz as a Candidate Gene
Christoph Fehr, Renee L. Shirley, John K. Belknap, John C. Crabbe, Kari J. Buck
J Neurosci. 2002 May 1; 22(9): 3730–3738. doi: 10.1523/JNEUROSCI.22-09-03730.2002
PMCID:
PMC6758356
AbstractArticlePDF–458K
17.
Thioesterase PPT1 balances viral resistance and efficient T cell crosspriming in dendritic cells
Pengju Ou, Lifen Wen, Xiaoli Liu, Jiancheng Huang, Xiaoling Huang, Chaofei Su, Ling Wang, Hai Ni, Boris Reizis, Cliff Y. Yang
J Exp Med. 2019 Sep 2; 216(9): 2091–2112. Published online 2019 Jul 1. doi: 10.1084/jem.20190041
PMCID:
PMC6719428
AbstractArticlePDF–5.2M
18.
A Role for Sensory end Organ-Derived Signals in Regulating Muscle Spindle Proprioceptor Phenotype
Dawei Wu, Ira Schieren, Yingzhi Qian, Chaolin Zhang, Thomas M. Jessell, Joriene C. de Nooij
J Neurosci. 2019 May 29; 39(22): 4252–4267. doi: 10.1523/JNEUROSCI.2671-18.2019
PMCID:
PMC6538853
AbstractArticlePDF–6.3M
19.
Developmental NMDA receptor dysregulation in the infantile neuronal ceroid lipofuscinosis mouse model
Kevin P Koster, Walter Francesconi, Fulvia Berton, Sami Alahmadi, Roshan Srinivas, Akira Yoshii
eLife. 2019; 8: e40316. Published online 2019 Apr 4. doi: 10.7554/eLife.40316
PMCID:
PMC6464704
AbstractArticlePDF–5.5M
20.
The Interactome of Palmitoyl-Protein Thioesterase 1 (PPT1) Affects Neuronal Morphology and Function
Tamar Sapir, Michal Segal, Gayane Grigoryan, Karin M. Hansson, Peter James, Menahem Segal, Orly Reiner
Front Cell Neurosci. 2019; 13: 92. Published online 2019 Mar 13. doi: 10.3389/fncel.2019.00092
PMCID:
PMC6424868
AbstractArticlePDF–4.8M

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