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Select item 106280981.

Acidified drinking water improves motor function, prevents tremors and changes disease trajectory in Cln2^R207X mice, a model of late infantile Batten disease

Attila D. Kovács, Jose L. Gonzalez Hernandez, David A. Pearce

Sci Rep. 2023; 13: 19229. Published online 2023 Nov 6. doi: 10.1038/s41598-023-46283-w

PMCID:: PMC10628098

Abstract Article PDF–1.9M

Select item 103306562.

A mouse mutant deficient in both neuronal ceroid lipofuscinosis-associated proteins CLN3 and TPP1

David E. Sleat, Whitney Banach-Petrosky, Katherine E. Larrimore, Yuliya Nemtsova, Jennifer A. Wiseman, Allison Najafi, Dymonn Johnson, Timothy A. Poole, Keigo Takahashi, Jonathan D. Cooper, Peter Lobel

J Inherit Metab Dis. Author manuscript; available in PMC 2024 Jul 1.

Published in final edited form as: J Inherit Metab Dis. 2023 Jul; 46(4): 720–734. Published online 2023 Apr 26. doi: 10.1002/jimd.12619

PMCID:: PMC10330656

Abstract Article PDF–1.7M

Select item 102667783.

Gene therapy ameliorates spontaneous seizures associated with cortical neuron loss in a Cln2^R207X mouse model

Keigo Takahashi, Elizabeth M. Eultgen, Sophie H. Wang, Nicholas R. Rensing, Hemanth R. Nelvagal, Joshua T. Dearborn, Olivier Danos, Nicholas Buss, Mark S. Sands, Michael Wong, Jonathan D. Cooper

J Clin Invest. 2023 Jun 15; 133(12): e165908. Published online 2023 Jun 15. doi: 10.1172/JCI165908

PMCID:: PMC10266778

Abstract Article PDF–3.9M

Select item 102521924.

Extracellular Vesicles Released by Genetically Modified Macrophages Activate Autophagy and Produce Potent Neuroprotection in Mouse Model of Lysosomal Storage Disorder, Batten Disease

Nazira El-Hage, Matthew J. Haney, Yuling Zhao, Myosotys Rodriguez, Zhanhong Wu, Mori Liu, Carson J. Swain, Hong Yuan, Elena V. Batrakova

Cells. 2023 Jun; 12(11): 1497. Published online 2023 May 29. doi: 10.3390/cells12111497

PMCID:: PMC10252192

Abstract Article PDF–4.1M

Select item 93089465.

Treadmill exercise reduces α-synuclein spreading via PPARα

Debashis Dutta, Ramesh Kumar Paidi, Sumita Raha, Avik Roy, Sujyoti Chandra, Kalipada Pahan

Cell Rep. Author manuscript; available in PMC 2022 Jul 24.

Published in final edited form as: Cell Rep. 2022 Jul 12; 40(2): 111058. doi: 10.1016/j.celrep.2022.111058

Correction in:: Cell Rep. 2024 Aug 27; 43(8): 114546.

PMCID:: PMC9308946

Abstract Article PDF–3.2M

Select item 92653446.

Mitoribosomal Deregulation Drives Senescence via TPP1-Mediated Telomere Deprotection

Seongki Min, So Mee Kwon, Jiwon Hong, Young-Kyoung Lee, Tae Jun Park, Su Bin Lim, Gyesoon Yoon

Cells. 2022 Jul; 11(13): 2079. Published online 2022 Jun 30. doi: 10.3390/cells11132079

PMCID:: PMC9265344

Abstract Article PDF–6.4M

Select item 85769197.

Brain transcriptome analysis of a CLN2 mouse model as a function of disease progression

Miriam S. Domowicz, Wen-Ching Chan, Patricia Claudio-Vázquez, Tatiana Gonzalez, Nancy B. Schwartz

J Neuroinflammation. 2021; 18: 262. Published online 2021 Nov 8. doi: 10.1186/s12974-021-02302-z

PMCID:: PMC8576919

Abstract Article PDF–4.0M

Select item 85482618.

Differential impact of a dyskeratosis congenita mutation in TPP1 on mouse hematopoiesis and germline

Jacqueline V Graniel, Kamlesh Bisht, Ann Friedman, James White, Eric Perkey, Ashley Vanderbeck, Alina Moroz, Léolène J Carrington, Joshua D Brandstadter, Frederick Allen, Adrienne Niederriter Shami, Peedikayil Thomas, Aniela Crayton, Mariel Manzor, Anna Mychalowych, Jennifer Chase, Saher S Hammoud, Catherine E Keegan, Ivan Maillard, Jayakrishnan Nandakumar

Life Sci Alliance. 2022 Jan; 5(1): e202101208. Published online 2021 Oct 13. doi: 10.26508/lsa.202101208

PMCID:: PMC8548261

Abstract Article PDF–3.5M

Select item 83132399.

Cell-Type-Specific Gene Expression in Developing Mouse Neocortex: Intermediate Progenitors Implicated in Axon Development

Francesco Bedogni, Robert F. Hevner

Front Mol Neurosci. 2021; 14: 686034. Published online 2021 Jul 12. doi: 10.3389/fnmol.2021.686034

PMCID:: PMC8313239

Abstract Article PDF–1.6M

Select item 826233710.

TPP1 mutagenesis screens unravel shelterin interfaces and functions in hematopoiesis

Sherilyn Grill, Shilpa Padmanaban, Ann Friedman, Eric Perkey, Frederick Allen, Valerie M. Tesmer, Jennifer Chase, Rami Khoriaty, Catherine E. Keegan, Ivan Maillard, Jayakrishnan Nandakumar

JCI Insight. 2021 May 10; 6(9): e138059. Published online 2021 May 10. doi: 10.1172/jci.insight.138059

PMCID:: PMC8262337

Abstract Article PDF–2.7M

Select item 793877011.

BraInMap elucidates the macromolecular connectivity landscape of mammalian brain

Reza Pourhaghighi, Peter E. A. Ash, Sadhna Phanse, Florian Goebels, Lucas Z. M. Hu, Siwei Chen, Yingying Zhang, Shayne D. Wierbowski, Samantha Boudeau, Mohamed T. Moutaoufik, Ramy H. Malty, Edyta Malolepsza, Kalliopi Tsafou, Aparna Nathan, Graham Cromar, Hongbo Guo, Ali Al Abdullatif, Daniel J. Apicco, Lindsay A. Becker, Aaron D. Gitler, Stefan M. Pulst, Ahmed Youssef, Ryan Hekman, Pierre C. Havugimana, Carl A. White, Benjamin C. Blum, Antonia Ratti, Camron D. Bryant, John Parkinson, Kasper Lage, Mohan Babu, Haiyuan Yu, Gary D. Bader, Benjamin Wolozin, Andrew Emili

Cell Syst. Author manuscript; available in PMC 2021 Apr 22.

Published in final edited form as: Cell Syst. 2020 Apr 22; 10(4): 333–350.e14. doi: 10.1016/j.cels.2020.03.003

Correction in:: Cell Syst. 2020 Aug 26; 11(2): 208.

PMCID:: PMC7938770

Abstract Article PDF–2.0M

Select item 673004912.

A Mouse Model of Classical Late-Infantile Neuronal Ceroid Lipofuscinosis Based on Targeted Disruption of the CLN2 Gene Results in a Loss of Tripeptidyl-Peptidase I Activity and Progressive Neurodegeneration

David E. Sleat, Jennifer A. Wiseman, Mukarram El-Banna, Kwi-Hye Kim, Qinwen Mao, Sandy Price, Shannon L. Macauley, Richard L. Sidman, Michael M. Shen, Qi Zhao, Marco A. Passini, Beverly L. Davidson, Gregory R. Stewart, Peter Lobel

J Neurosci. 2004 Oct 13; 24(41): 9117–9126. doi: 10.1523/JNEUROSCI.2729-04.2004

PMCID:: PMC6730049

Abstract Article PDF–1.3M

Select item 658849213.

Upregulation of tripeptidyl-peptidase 1 by 3-hydroxy-(2,2)-dimethyl butyrate, a brain endogenous ligand of PPARα: Implications for late-infantile Batten disease therapy

Sudipta Chakrabarti, Sujyoti Chandra, Avik Roy, Sridevi Dasarathi, Madhuchhanda Kundu, Kalipada Pahan

Neurobiol Dis. Author manuscript; available in PMC 2020 Jul 1.

Published in final edited form as: Neurobiol Dis. 2019 Jul; 127: 362–373. Published online 2019 Mar 28. doi: 10.1016/j.nbd.2019.03.025

Correction in:: Neurobiol Dis. 2024 Mar 27 : 106479.

PMCID:: PMC6588492

Abstract Article PDF–2.5M

Select item 607255914.

53BP1/Rif1/Shieldin counteract DSB resection through CST/Polα-dependent fill-in

Zachary Mirman, Francisca Lottersberger, Hiroyuki Takai, Tatsuya Kibe, Yi Gong, Kaori Takai, Alessandro Bianchi, Michal Zimmermann, Daniel Durocher, Titia de Lange

Nature. Author manuscript; available in PMC 2019 Jan 18.

Published in final edited form as: Nature. 2018 Aug; 560(7716): 112–116. Published online 2018 Jul 18. doi: 10.1038/s41586-018-0324-7

PMCID:: PMC6072559

Abstract Article PDF–1.8M

Select item 580069815.

Inducible transgenic expression of tripeptidyl peptidase 1 in a mouse model of late-infantile neuronal ceroid lipofuscinosis

Yuliya Nemtsova, Jennifer A. Wiseman, Mukarram El-Banna, Peter Lobel, David E. Sleat

PLoS One. 2018; 13(2): e0192286. Published online 2018 Feb 6. doi: 10.1371/journal.pone.0192286

PMCID:: PMC5800698

Abstract Article PDF–4.3M

Select item 555886116.

Loss of TMEM106B Ameliorates Lysosomal and Frontotemporal Dementia-Related Phenotypes in Progranulin-Deficient Mice

Zoe A. Klein, Hideyuki Takahashi, Mengxiao Ma, Massimiliano Stagi, Melissa Zhou, TuKiet T. Lam, Stephen M. Strittmatter

Neuron. Author manuscript; available in PMC 2018 Jul 19.

Published in final edited form as: Neuron. 2017 Jul 19; 95(2): 281–296.e6. doi: 10.1016/j.neuron.2017.06.026

PMCID:: PMC5558861

Abstract Article PDF–2.3M

Select item 541305917.

A tailored mouse model of CLN2 disease: A nonsense mutant for testing personalized therapies

Ryan D. Geraets, Logan M. Langin, Jacob T. Cain, Camille M. Parker, Rosanna Beraldi, Attila D. Kovacs, Jill M. Weimer, David A. Pearce

PLoS One. 2017; 12(5): e0176526. Published online 2017 May 2. doi: 10.1371/journal.pone.0176526

PMCID:: PMC5413059

Abstract Article PDF–16M

Select item 539532718.

Gemfibrozil, Food and Drug Administration-approved lipid-lowering drug, increases longevity in mouse model of Late Infantile Neuronal Ceroid Lipofuscinosis

Arunava Ghosh, Suresh Babu Rangasamy, Khushbu K. Modi, Kalipada Pahan

J Neurochem. Author manuscript; available in PMC 2018 May 1.

Published in final edited form as: J Neurochem. 2017 May; 141(3): 423–435. Published online 2017 Apr 3. doi: 10.1111/jnc.13987

PMCID:: PMC5395327

Abstract Article PDF–3.1M

Select item 495013719.

Data on characterizing the gene expression patterns of neuronal ceroid lipofuscinosis genes: CLN1, CLN2, CLN3, CLN5 and their association to interneuron and neurotransmission markers: Parvalbumin and Somatostatin

Helena M. Minye, Anna-Liisa Fabritius, Jouni Vesa, Leena Peltonen

Data Brief. 2016 Sep; 8: 741–749. Published online 2016 Jun 23. doi: 10.1016/j.dib.2016.06.027

PMCID:: PMC4950137

Abstract Article PDF–4.5M

Select item 489981620.

Antigen presenting cell abnormalities in the Cln3^−/− mouse model of juvenile neuronal ceroid lipofuscinosis

Samantha L. Hersrud, Attila D. Kovács, David A. Pearce

Biochim Biophys Acta. Author manuscript; available in PMC 2017 Jul 1.

Published in final edited form as: Biochim Biophys Acta. 2016 Jul; 1862(7): 1324–1336. Published online 2016 Apr 19. doi: 10.1016/j.bbadis.2016.04.011

PMCID:: PMC4899816

Abstract Article PDF–1.3M

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