R-Roscovitine Improves Motoneuron Function in Mouse Models for Spinal Muscular Atrophy

Authors: Tejero, Rocio, Balk, Stefanie, Franco-Espin, Julio, Ojeda, Jorge, Hennlein, Luisa, Drexl, Hans, Dombert, Benjamin, Clausen, Jan-Dierk, Torres-Benito, Laura, Saal-Bauernschubert, Lena, Blum, Robert, Briese, Michael, Appenzeller, Silke, Tabares, Lucia, Jablonka, Sibylle

Source: In iScience 21 February 2020 23(2)

Impaired Local Translation of β-actin mRNA in Ighmbp2-Deficient Motoneurons: Implications for Spinal Muscular Atrophy with respiratory Distress (SMARD1)

Authors: Surrey, Verena, Zöller, Caren, Lork, Alicia Andrea, Moradi, Mehri, Balk, Stefanie, Dombert, Benjamin, Saal-Bauernschubert, Lena, Briese, Michael, Appenzeller, Silke, Fischer, Utz, Jablonka, Sibylle

Source: In Neuroscience 21 August 2018 386:24-40

Smn, the Spinal Muscular Atrophy-Determining Gene Product, Modulates Axon Growth and Localization of β-Actin mRNA in Growth Cones of Motoneurons

Authors: Rossoll, Wilfried, Jablonka, Sibylle, Andreassi, Catia, Kröning, Ann-Kathrin, Karle, Kathrin, Monani, Umrao R., Sendtner, Michael

Source: The Journal of Cell Biology, 2003 Nov . 163(4), 801-812.

Access URL: https://www.jstor.org/stable/1621871

A Transgene Carrying an A2G Missense Mutation in the SMN Gene Modulates Phenotypic Severity in Mice with Severe (Type I) Spinal Muscular Atrophy

Authors: Monani, Umrao R., Pastore, Matthew T., Gavrilina, Tatiana O., Jablonka, Sibylle, Le, Thanh T., Andreassi, Catia, DiCocco, Jennifer M., Lorson, Christian, Androphy, Elliot J., Sendtner, Michael, Podell, Michael

Source: The Journal of Cell Biology, 2003 Jan 01. 160(1), 41-52.

Access URL: https://www.jstor.org/stable/1621393

Missense Mutation in the Tubulin-Specific Chaperone E (Tbce) Gene in the Mouse Mutant Progressive Motor Neuronopathy, a Model of Human Motoneuron Disease

Authors: Bömmel, Heike, Xie, Gang, Rossoll, Wilfried, Wiese, Stefan, Jablonka, Sibylle, Boehm, Thomas, Sendtner, Michael

Source: The Journal of Cell Biology, 2002 Nov . 159(4), 563-569.

Access URL: https://www.jstor.org/stable/1621310

Gene Targeting of Gemin2 in Mice Reveals a Correlation between Defects in the Biogenesis of U snRNPs and Motoneuron Cell Death

Authors: Jablonka, Sibylle, Holtmann, Bettina, Meister, Gunter, Bandilla, Michael, Rossoll, Wilfried, Fischer, Utz, Sendtner, Michael

Source: Proceedings of the National Academy of Sciences of the United States of America, 2002 Jul . 99(15), 10126-10131.

Access URL: https://www.jstor.org/stable/3059345

Disease Mechanisms and Therapeutic Approaches in SMARD1—Insights from Animal Models and Cell Models.

Authors: Jablonka, Sibylle, Yildirim, Ezgi

Source: Biomedicines; Apr2024, Vol. 12 Issue 4, p845, 17p

Subject Terms: SPINAL muscular atrophy, THERAPEUTICS, ANIMAL models in research, RNA helicase, MUSCLE weakness, GLYCOGEN storage disease type II

RNA helicase IGHMBP2 regulates THO complex to ensure cellular mRNA homeostasis

Authors: Prusty, Archana Bairavasundaram, Hirmer, Anja, Sierra-Delgado, Julieth Andrea, Huber, Hannes, Guenther, Ulf-Peter, Schlosser, Andreas, Dybkov, Olexandr, Yildirim, Ezgi, Urlaub, Henning, Meyer, Kathrin C., Jablonka, Sibylle, Erhard, Florian, Fischer, Utz

Source: In Cell Reports 27 February 2024 43(2)

The SMA Modifier Plastin 3 Targets Cell Membrane-Associated Proteins in Motoneurons.

Authors: Jablonka, Sibylle¹ (AUTHOR) Jablonka_S@ukw.de, Schäfer, Natascha¹ (AUTHOR)

Source: Neuroscience Insights. 1/19/2024, p1-4. 4p.

Subject Terms: *CELL receptors, *CALCIUM channels, *SPINAL muscular atrophy, *MEMBRANE proteins, *NEUROMUSCULAR diseases, *MYONEURAL junction, *MOTOR neurons

Cochlear nucleus whole mount explants promote the differentiation of neuronal stem cells from the cochlear nucleus in co-culture experiments

Authors: Rak, Kristen, Völker, Johannes, Jürgens, Lukas, Völker, Christine, Frenz, Silke, Scherzad, Agmal, Schendzielorz, Philipp, Jablonka, Sibylle, Mlynski, Robert, Radeloff, Andreas, Hagen, Rudolf

Source: In Brain Research 7 August 2015 1616:58-70

Mutation of the TBCE gene causes disturbance of microtubules in the auditory nerve and cochlear outer hair cell degeneration accompanied by progressive hearing loss in the pmn/pmn mouse

Authors: Rak, Kristen, Frenz, Silke, Radeloff, Andreas, Groh, Janos, Jablonka, Sibylle, Martini, Rudolf, Hagen, Rudolf, Mlynski, Robert

Source: In Experimental Neurology December 2013 250:333-340

Fast motor axon loss in SMARD1 does not correspond to morphological and functional alterations of the NMJ

Authors: Krieger, Frank, Elflein, Nicole, Ruiz, Rocio, Guerra, Joana, Serrano, Antonio L., Asan, Esther, Tabares, Lucia, Jablonka, Sibylle

Source: In Neurobiology of Disease June 2013 54:169-182

Loss of Tdp-43 disrupts the axonal transcriptome of motoneurons accompanied by impaired axonal translation and mitochondria function

Authors: Briese, Michael, Saal-Bauernschubert, Lena, Lüningschrör, Patrick, Moradi, Mehri, Dombert, Benjamin, Surrey, Verena, Appenzeller, Silke, Deng, Chunchu, Jablonka, Sibylle, Sendtner, Michael

Source: Acta neuropathologica communications. 8(1)

Availability: http://explore.bl.uk/primo_library/libweb/action/display.do?tabs=detailsTab&gathStatTab=true&ct=display&fn=search&doc=ETOCvdc_100172013532.0x000001&indx=1&recIds=ETOCvdc_100172013532.0x000001

Mosaic pattern of Cre recombinase expression in cochlear outer hair cells of the Brn3.1 Cre mouse

Authors: Frenz, Silke, Rak, Kristen, Völker, Johannes, Jürgens, Lukas, Scherzad, Agmal, Schendzielorz, Philipp, Radeloff, Andreas, Jablonka, Sibylle, Hansen, Stefan, Mlynski, Robert, Hagen, Rudolf

Source: NeuroReport. 26(6):309-313

Availability: http://explore.bl.uk/primo_library/libweb/action/display.do?tabs=detailsTab&gathStatTab=true&ct=display&fn=search&doc=ETOCvdc_100036947344.0x000001&indx=1&recIds=ETOCvdc_100036947344.0x000001

CDNF rescues motor neurons in models of amyotrophic lateral sclerosis by targeting endoplasmic reticulum stress.

Authors: Lorenzo, Francesca De^1,2, Lüningschrör, Patrick³, Nam, Jinhan^1,2, Beckett, Liam^1,2, Pilotto, Federica⁴, Galli, Emilia¹, Lindholm, Päivi¹, Collenberg, Cora Rüdt von³, Mungwa, Simon Tii³, Jablonka, Sibylle³, Kauder, Julia⁵, Thau-Habermann, Nadine⁵, Petri, Susanne⁵, Lindholm, Dan^6,7, Saxena, Smita⁴, Sendtner, Michael³, Saarma, Mart¹, Voutilainen, Merja H^1,2, merja.h.voutilainen@helsinki.fi

Source: Brain: A Journal of Neurology; Sep2023, Vol. 146 Issue 9, p3783-3799, 17p

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Therapeutic effects of PEGylated insulin-like growth factor I in the pmn mouse model of motoneuron disease

Authors: Jablonka, Sibylle, Holtmann, Bettina, Sendtner, Michael, Metzger, Friedrich

Source: In Experimental Neurology December 2011 232(2):261-269

Adenosine Receptor ${\rm A}_{2{\rm A}}\text{-}{\rm R}$ Contributes to Motoneuron Survival by Transactivating the Tyrosine Kinase Receptor TrkB

Authors: Wiese, Stefan, Jablonka, Sibylle, Holtmann, Bettina, Orel, Nadiya, Rajagopal, Rithwick, Chao, Moses V., Sendtner, Michael

Source: Proceedings of the National Academy of Sciences of the United States of America, 2007 Oct . 104(43), 17210-17215.

Access URL: https://www.jstor.org/stable/25450207

Defective Ca²⁺ Channel Clustering in Axon Terminals Disturbs Excitability in Motoneurons in Spinal Muscular Atrophy

Authors: Jablonka, Sibylle, Beck, Marcus, Lechner, Barbara Dorothea, Mayer, Christine, Sendtner, Michael

Source: The Journal of Cell Biology, 2007 Oct 01. 179(1), 139-149.

Access URL: https://www.jstor.org/stable/30050447

Nifedipine Ameliorates Cellular Differentiation Defects of Smn-Deficient Motor Neurons and Enhances Neuromuscular Transmission in SMA Mice.

Authors: Tejero, Rocio, Alsakkal, Mohammad, Hennlein, Luisa, Lopez-Cabello, Ana M., Jablonka, Sibylle, Tabares, Lucia

Source: International Journal of Molecular Sciences; Apr2023, Vol. 24 Issue 8, p7648, 17p

Subject Terms: MOTOR neurons, NEUROMUSCULAR transmission, SPINAL muscular atrophy, NIFEDIPINE, NERVE endings, MOTOR neuron diseases, NEURAL transmission