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Select item 107644941.

In depth behavioral phenotyping unravels complex motor disturbances in Cstb^−/− mouse, a model for progressive myoclonus epilepsy type 1

Eveliina Pollari, Saara Tegelberg, Harry Björklund, Reetta Kälviäinen, Anna-Elina Lehesjoki, Antti Haapalinna

Front Behav Neurosci. 2023; 17: 1325051. Published online 2023 Dec 21. doi: 10.3389/fnbeh.2023.1325051

PMCID:: PMC10764494

Article PDF–1.1M

Select item 102132082.

Progressive mitochondrial dysfunction in cerebellar synaptosomes of cystatin B-deficient mice

Katarin Gorski, Christopher B. Jackson, Tuula A. Nyman, Veronika Rezov, Brendan J. Battersby, Anna-Elina Lehesjoki

Front Mol Neurosci. 2023; 16: 1175851. Published online 2023 May 12. doi: 10.3389/fnmol.2023.1175851

PMCID:: PMC10213208

Article PDF–3.6M

Select item 76916383.

Quantitative Changes in the Mitochondrial Proteome of Cerebellar Synaptosomes From Preclinical Cystatin B-Deficient Mice

Katarin Gorski, Albert Spoljaric, Tuula A. Nyman, Kai Kaila, Brendan J. Battersby, Anna-Elina Lehesjoki

Front Mol Neurosci. 2020; 13: 570640. Published online 2020 Nov 13. doi: 10.3389/fnmol.2020.570640

PMCID:: PMC7691638

Article PDF–2.1M

Select item 51270534.

Brain inflammation is accompanied by peripheral inflammation in Cstb^−/− mice, a model for progressive myoclonus epilepsy

Olesya Okuneva, Zhilin Li, Inken Körber, Saara Tegelberg, Tarja Joensuu, Li Tian, Anna-Elina Lehesjoki

J Neuroinflammation. 2016; 13: 298. Published online 2016 Nov 28. doi: 10.1186/s12974-016-0764-7

PMCID:: PMC5127053

Article PDF–2.3M

Select item 49270945.

Gene-Expression Profiling Suggests Impaired Signaling via the Interferon Pathway in Cstb^-/- Microglia

Inken Körber, Shintaro Katayama, Elisabet Einarsdottir, Kaarel Krjutškov, Paula Hakala, Juha Kere, Anna-Elina Lehesjoki, Tarja Joensuu

PLoS One. 2016; 11(6): e0158195. Published online 2016 Jun 29. doi: 10.1371/journal.pone.0158195

PMCID:: PMC4927094

Article PDF–2.5M

Select item 42316536.

A Role for Stefin B (Cystatin B) in Inflammation and Endotoxemia

Katarina Maher, Barbara Jerič Kokelj, Miha Butinar, Georgy Mikhaylov, Mateja Manček-Keber, Veronika Stoka, Olga Vasiljeva, Boris Turk, Sergei A. Grigoryev, Nataša Kopitar-Jerala

J Biol Chem. 2014 Nov 14; 289(46): 31736–31750. Published online 2014 Oct 6. doi: 10.1074/jbc.M114.609396

PMCID:: PMC4231653

Article PDF–3.2M

Select item 39483517.

Progressive Volume Loss and White Matter Degeneration in Cstb-Deficient Mice: A Diffusion Tensor and Longitudinal Volumetry MRI Study

Otto Manninen, Teemu Laitinen, Kimmo K. Lehtimäki, Saara Tegelberg, Anna-Elina Lehesjoki, Olli Gröhn, Outi Kopra

PLoS One. 2014; 9(3): e90709. Published online 2014 Mar 6. doi: 10.1371/journal.pone.0090709

PMCID:: PMC3948351

Article PDF–1.8M

Select item 39373338.

Gene Expression Alterations in the Cerebellum and Granule Neurons of Cstb^−/− Mouse Are Associated with Early Synaptic Changes and Inflammation

Tarja Joensuu, Saara Tegelberg, Eva Reinmaa, Mikael Segerstråle, Paula Hakala, Heidi Pehkonen, Esa R. Korpi, Jaana Tyynelä, Tomi Taira, Iiris Hovatta, Outi Kopra, Anna-Elina Lehesjoki

PLoS One. 2014; 9(2): e89321. Published online 2014 Feb 27. doi: 10.1371/journal.pone.0089321

PMCID:: PMC3937333

Article PDF–1.4M

Select item 32275739.

Characterization of PTZ-Induced Seizure Susceptibility in a Down Syndrome Mouse Model That Overexpresses CSTB

Véronique Brault, Benoît Martin, Nathalie Costet, Jean-Charles Bizot, Yann Hérault

PLoS One. 2011; 6(11): e27845. Published online 2011 Nov 30. doi: 10.1371/journal.pone.0027845

PMCID:: PMC3227573

Article PDF–506K

Select item 829777310.

The effects of Cstb duplication on APP/amyloid-β pathology and cathepsin B activity in a mouse model

Yixing Wu, Heather T. Whittaker, Suzanna Noy, Karen Cleverley, Veronique Brault, Yann Herault, Elizabeth M. C. Fisher, Frances K. Wiseman

PLoS One. 2021; 16(7): e0242236. Published online 2021 Jul 22. doi: 10.1371/journal.pone.0242236

PMCID:: PMC8297773

Article PDF–1.6M

Select item 410546311.

Human Stefin B Role in Cell's Response to Misfolded Proteins and Autophagy

Mira Polajnar, Tina Zavašnik-Bergant, Katja Škerget, Matej Vizovišek, Robert Vidmar, Marko Fonović, Nataša Kopitar-Jerala, Uroš Petrovič, Susanna Navarro, Salvador Ventura, Eva Žerovnik

PLoS One. 2014; 9(7): e102500. Published online 2014 Jul 21. doi: 10.1371/journal.pone.0102500

PMCID:: PMC4105463

Article PDF–4.6M

Select item 359045212.

The telomeric part of the human chromosome 21 from Cstb to Prmt2 is not necessary for the locomotor and short-term memory deficits observed in the Tc1 mouse model of Down syndrome

Arnaud Duchon, Stéphanie Pothion, Véronique Brault, Andrew J. Sharp, Victor L.J. Tybulewicz, Elizabeth M.C. Fisher, Yann Herault

Behav Brain Res. 2011 Mar 1; 217(2): 271–281. doi: 10.1016/j.bbr.2010.10.023

PMCID:: PMC3590452

Article

Select item 296678513.

Cystatin C Rescues Degenerating Neurons in a Cystatin B-Knockout Mouse Model of Progressive Myoclonus Epilepsy

Gurjinder Kaur, Panaiyur Mohan, Monika Pawlik, Steven DeRosa, Jay Fajiculay, Shaoli Che, Anders Grubb, Stephen D. Ginsberg, Ralph A. Nixon, Efrat Levy

Am J Pathol. 2010 Nov; 177(5): 2256–2267. doi: 10.2353/ajpath.2010.100461

PMCID:: PMC2966785

Article PDF–1.4M

Select item 269449514.

Cystatin B Deficiency Sensitizes Neurons to Oxidative Stress in Progressive Myoclonus Epilepsy, EPM1

Maria K. Lehtinen, Saara Tegelberg, Hyman Schipper, Haixiang Su, Hillel Zukor, Otto Manninen, Outi Kopra, Tarja Joensuu, Paula Hakala, Azad Bonni, Anna-Elina Lehesjoki

J Neurosci. 2009 May 6; 29(18): 5910–5915. doi: 10.1523/JNEUROSCI.0682-09.2009

PMCID:: PMC2694495

Article PDF–232K

Select item 253570115.

Inducing Segmental Aneuploid Mosaicism in the Mouse Through Targeted Asymmetric Sister Chromatid Event of Recombination

Arnaud Duchon, Vanessa Besson, Patricia Lopes Pereira, Laetitia Magnol, Yann Hérault

Genetics. 2008 Sep; 180(1): 51–59. doi: 10.1534/genetics.108.092312

PMCID:: PMC2535701

Article PDF–1.6M

Select item 727854716.

Cystatin B is essential for proliferation and interneuron migration in individuals with EPM1 epilepsy

Francesco Di Matteo, Fabrizia Pipicelli, Christina Kyrousi, Isabella Tovecci, Eduardo Penna, Marianna Crispino, Angela Chambery, Rosita Russo, Ane Cristina Ayo‐Martin, Martina Giordano, Anke Hoffmann, Emilio Ciusani, Laura Canafoglia, Magdalena Götz, Rossella Di Giaimo, Silvia Cappello

EMBO Mol Med. 2020 Jun 8; 12(6): e11419. Published online 2020 May 7. doi: 10.15252/emmm.201911419

PMCID:: PMC7278547

Article PDF–4.6M

Select item 478790917.

Genetic and pharmacological evidence implicates cathepsins in Niemann-Pick C cerebellar degeneration

Chan Chung, Prasanth Puthanveetil, Daniel S. Ory, Andrew P. Lieberman

Hum Mol Genet. 2016 Apr 1; 25(7): 1434–1446. Published online 2016 Jan 28. doi: 10.1093/hmg/ddw025

PMCID:: PMC4787909

Article PDF–1.2M

Select item 5851718.

Genomic profiling of short- and long-term caloric restriction effects in the liver of aging mice

Shelley X. Cao, Joseph M. Dhahbi, Patricia L. Mote, Stephen R. Spindler

Proc Natl Acad Sci U S A. 2001 Sep 11; 98(19): 10630–10635. Published online 2001 Sep 4. doi: 10.1073/pnas.191313598

PMCID:: PMC58517

Article PDF–114K

Select item 284317019.

Stefin B Interacts with Histones and Cathepsin L in the Nucleus

Slavko Čeru, Špela Konjar, Katarina Maher, Urška Repnik, Igor Križaj, Mojca Benčina, Miha Renko, Alain Nepveu, Eva Žerovnik, Boris Turk, Nataša Kopitar-Jerala

J Biol Chem. 2010 Mar 26; 285(13): 10078–10086. Published online 2010 Jan 14. doi: 10.1074/jbc.M109.034793