Research output per year
Research output per year
- 17 results
Search results
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2023
In depth behavioral phenotyping unravels complex motor disturbances in Cstb-/- mouse, a model for progressive myoclonus epilepsy type 1
Pollari, E., Tegelberg, S., Björklund, H., Kälviäinen, R., Lehesjoki, A.-E. & Haapalinna, A., 21 Dec 2023, In: Frontiers in Behavioral Neuroscience. 17Research output: Contribution to journal › Article › Scientific › peer-review
Open AccessFile -
2022
Cystatin B deficiency results in sustained histone H3 tail cleavage in postnatal mouse brain mediated by increased chromatin-associated cathepsin L activity
Daura, E., Tegelberg, S., Hakala, P., Lehesjoki, A.-E. & Joensuu, T., 30 Nov 2022, In: Frontiers in Molecular Neuroscience. 15, 13 p., 1069122.Research output: Contribution to journal › Article › Scientific › peer-review
Open AccessFile -
2021
Cystatin B-deficiency triggers ectopic histone H3 tail cleavage during neurogenesis
Daura, E., Tegelberg, S., Yoshihara, M., Jackson, C., Simonetti, F., Aksentjeff, K., Ezer, S., Hakala, P., Katayama, S., Kere, J., Lehesjoki, A.-E. & Joensuu, T., Aug 2021, In: Neurobiology of Disease. 156, 13 p., 105418.Research output: Contribution to journal › Article › Scientific › peer-review
Open AccessFile -
2020
Microglial phagocytosis dysfunction in the dentate gyrus is related to local neuronal activity in a genetic model of epilepsy
Sierra-Torre, V., Plaza-Zabala, A., Bonifazi, P., Abiega, O., Diaz-Aparicio, I., Tegelberg, S., Lehesjoki, A.-E., Valero, J. & Sierra, A., Nov 2020, In: Epilepsia. 61, 11, p. 2593-2608 16 p.Research output: Contribution to journal › Article › Scientific › peer-review
Open AccessFile -
2019
Alternative oxidase-mediated respiration prevents lethal mitochondrial cardiomyopathy
Rajendran, J., Purhonen, J., Tegelberg, S., Smolander, O.-P., Mörgelin, M., Rozman, J., Gailus-Durner, V., Fuchs, H., de Angelis, M. H., Auvinen, P., Mervaala, E., Jacobs, H. T., Szibor, M., Fellman, V. & Kallijärvi, J., Jan 2019, In: EMBO molecular medicine. 11, 1, 19 p., 9456.Research output: Contribution to journal › Article › Scientific › peer-review
Open AccessFile -
2018
NAD(+) repletion produces no therapeutic effect in mice with respiratory chain complex III deficiency and chronic energy deprivation
Purhonen, J., Rajendran, J., Tegelberg, S., Smolander, O.-P., Pirinen, E., Kallijärvi, J. & Fellman, V., Nov 2018, In: FASEB Journal. 32, 11, p. 5913-5926 14 p.Research output: Contribution to journal › Article › Scientific › peer-review
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2017
Respiratory chain complex III deficiency due to mutated BCS1L: a novel phenotype with encephalomyopathy, partially phenocopied in a Bcs1l mutant mouse model
Tegelberg, S., Tomasic, N., Kallijärvi, J., Purhonen, J., Elmer, E., Lindberg, E., Nord, D. G., Soller, M., Lesko, N., Wedell, A., Bruhn, H., Freyer, C., Stranneheim, H., Wibom, R., Nennesmo, I., Wredenberg, A., Eklund, E. A. & Fellman, V., 20 Apr 2017, In: Orphanet journal of rare diseases. 12, 14 p., 73.Research output: Contribution to journal › Article › Scientific › peer-review
Open AccessFile -
ZNHIT3 is defective in PEHO syndrome, a severe encephalopathy with cerebellar granule neuron loss
Anttonen, A.-K., Laari, A., Kousi, M., Yang, Y. J., Jääskeläinen, T., Somer, M., Siintola, E., Jakkula, E., Muona, M., Tegelberg, S., Lönnqvist, T., Pihko, H., Valanne, L., Paetau, A., Lun, M. P., Hästbacka, J., Kopra, O., Joensuu, T., Katsanis, N. & Lehtinen, M. K. & 2 others, , May 2017, In: Brain : a journal of neurology. 140, p. 1267-1279 13 p.Research output: Contribution to journal › Article › Scientific › peer-review
Open AccessFile -
2016
Brain inflammation is accompanied by peripheral inflammation in Cstb(-/-) mice, a model for progressive myoclonus epilepsy
Okuneva, O., Li, Z., Korber, I., Tegelberg, S., Joensuu, T., Tian, L. & Lehesjoki, A.-E., 28 Nov 2016, In: Journal of Neuroinflammation. 13, 10 p., 298.Research output: Contribution to journal › Article › Scientific › peer-review
Open AccessFile -
2014
Gene Expression Alterations in the Cerebellum and Granule Neurons of Cstb(-/-) Mouse Are Associated with Early Synaptic Changes and Inflammation
Joensuu, T., Tegelberg, S., Reinmaa, E., Segerstrale, M., Hakala, P., Pehkonen, H., Korpi, E. R., Vesterinen, J., Taira, T., Hovatta, I., Kopra, O. & Lehesjoki, A.-E., 27 Feb 2014, In: PLoS One. 9, 2, 12 p., 89321.Research output: Contribution to journal › Article › Scientific › peer-review
Open AccessFile -
Progressive Volume Loss and White Matter Degeneration in Cstb-Deficient Mice: A Diffusion Tensor and Longitudinal Volumetry MRI Study
Manninen, O., Laitinen, T., Lehtimaki, K. K., Tegelberg, S., Lehesjoki, A.-E., Grohn, O. & Kopra, O., 6 Mar 2014, In: PLoS One. 9, 3, 7 p.Research output: Contribution to journal › Article › Scientific › peer-review
Open AccessFile -
2013
Early neuropathological changes in the mouse model for progressive myoclonus epilepsy of Unverricht-Lundborg type, EPM1
Tegelberg, S., 2013, Helsinki: [S. Tegelberg]. 107 p.Research output: Thesis › Doctoral Thesis › Collection of Articles
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2012
Early Microglial Activation Precedes Neuronal Loss in the Brain of the Cstb(-/-) Mouse Model of Progressive Myoclonus Epilepsy, EPM1
Tegelberg, S., Kopra, O., Joensuu, T., Cooper, J. D. & Lehesjoki, A.-E., 2012, In: Journal of Neuropathology and Experimental Neurology. 71, 1, p. 40-53 14 p.Research output: Contribution to journal › Article › Scientific › peer-review
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2009
Cystatin B deficiency sensitizes neurons to oxidative stress in progressive myoclonus epilepsy, EPM1
Lehtinen, M. K., Tegelberg, S., Schipper, H., Su, H., Zukor, H., Manninen, O., Kopra, O., Joensuu, T. H., Hakala, P., Bonni, A. & Lehesjoki, A.-E., 2009, In: Journal of Neuroscience. 29, 18, p. 5910-5915 6 p.Research output: Contribution to journal › Article › Scientific › peer-review
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2007
Cystatin B: mutation detection, alternative splicing and expression in progressive myclonus epilepsy of Unverricht-Lundborg type (EPM1) patients
Joensuu, T., Kuronen, M., Alakurtti, K., Tegelberg, S., Hakala, P., Aalto, A., Huopaniemi, L., Aula, N., Michellucci, R., Eriksson, K. & Lehesjoki, A.-E., 2007, In: European Journal of Human Genetics. 15, 2, p. 185-193 9 p.Research output: Contribution to journal › Article › Scientific › peer-review
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2004
Variant late infantile neuronal ceroid lipofuscinosis in a subset of Turkish patients is allelic to northern epilepsy
Ranta, S., Topcu, M., Tegelberg, S., Tan, H., Ustubutun, A., Saatci, I., Dufke, A., Enders, H., Pohl, K., Alembik, Y., Mitchell, W. A., Mole, S. E. & Lehesjoki, A. E., 2004, In: Human mutation. 23, p. 300-305 6 p.Research output: Contribution to journal › Article › Scientific › peer-review
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2001
Mutations in a novel gene with transmembrane domains underlie Usher syndrome type 3
Joensuu, T., Hamalainen, R., Yuan, B., Johnson, C., Tegelberg, S., Gasparini, P., Zelante, L., Pirvola, U., Pakarinen, L., Lehesjoki, A. E., de la Chapelle, A. & Sankila, E. M., 2001, In: American Journal of Human Genetics. 69, p. 673-684 12 p.Research output: Contribution to journal › Article › Scientific › peer-review