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Alejandro F. De Nicola, María Meyer, Laura Garay, Maria Sol Kruse, Michael Schumacher, Rachida Guennoun & Maria Claudia Gonzalez Deniselle. (2021) Progesterone and Allopregnanolone Neuroprotective Effects in the Wobbler Mouse Model of Amyotrophic Lateral Sclerosis. Cellular and Molecular Neurobiology 42:1, pages 23-40.
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Jian Wei, Ying-Yu Zhang, Jie Luo, Ju-Qiong Wang, Yu-Xia Zhou, Hong-Hua Miao, Xiong-Jie Shi, Yu-Xiu Qu, Jie Xu, Bo-Liang Li & Bao-Liang Song. (2017) The GARP Complex Is Involved in Intracellular Cholesterol Transport via Targeting NPC2 to Lysosomes. Cell Reports 19:13, pages 2823-2835.
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Carolin Dahlke, Darius Saberi, Bastian Ott, Beate Brand-Saberi, Thomas Schmitt-John & Carsten Theiss. (2015) Inflammation and neuronal death in the motor cortex of the wobbler mouse, an ALS animal model. Journal of Neuroinflammation 12:1.
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Thomas Schmitt-John. (2015) VPS54 and the wobbler mouse. Frontiers in Neuroscience 9.
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Ashley R. Jones, Claire Troakes, Andrew King, Vibhu Sahni, Simone De Jong, Koen Bossers, Efterpi Papouli, Muddassar Mirza, Safa Al-Sarraj, Christopher E. Shaw, Pamela J. Shaw, Janine Kirby, Jan H. Veldink, Jeffrey D. Macklis, John F. Powell & Ammar Al-Chalabi. (2015) Stratified gene expression analysis identifies major amyotrophic lateral sclerosis genes. Neurobiology of Aging 36:5, pages 2006.e1-2006.e9.
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Ashling Holland, Thomas Schmitt-John, Paul Dowling, Paula Meleady, Michael Henry, Martin Clynes & Kay Ohlendieck. (2014) Intricate effects of primary motor neuronopathy on contractile proteins and metabolic muscle enzymes as revealed by label-free mass spectrometry. Bioscience Reports 34:4.
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Harald Jockusch, Ashling Holland, Lisa Staunton, Thomas Schmitt-John, Peter Heimann, Paul Dowling & Kay Ohlendieck. (2014) Pathoproteomics of testicular tissue deficient in the GARP component VPS54: The wobbler mouse model of globozoospermia. PROTEOMICS 14:7-8, pages 839-852.
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Marco Peviani, Eleonora Salvaneschi, Leonardo Bontempi, Alessandro Petese, Antonio Manzo, Daniela Rossi, Mario Salmona, Simona Collina, Paolo Bigini & Daniela Curti. (2014) Neuroprotective effects of the Sigma-1 receptor (S1R) agonist PRE-084, in a mouse model of motor neuron disease not linked to SOD1 mutation. Neurobiology of Disease 62, pages 218-232.
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Chiara Paiardi, Maria Enrica Pasini, Alida Amadeo, Mariarosa Gioria & Giovanna Berruti. (2013) The ESCRT-deubiquitinating enzyme USP8 in the cervical spinal cord of wild-type and Vps54-recessive (wobbler) mutant mice. Histochemistry and Cell Biology 141:1, pages 57-73.
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Jakob Maximilian Moser, Paolo Bigini & Thomas Schmitt-John. (2013) The wobbler mouse, an ALS animal model. Molecular Genetics and Genomics 288:5-6, pages 207-229.
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Páll Karlsson, Aida Droce, Jakob Moser, Simon Cuhlmann, Carolina Padilla, Peter Heimann, Jörg Bartsch, Annette Füchtbauer, Ernst-Martin Füchtbauer & Thomas Schmitt-John. (2013) Loss of Vps54 Function Leads to Vesicle Traffic Impairment, Protein Mis-Sorting and Embryonic Lethality. International Journal of Molecular Sciences 14:6, pages 10908-10925.
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Francesca DiFebo, Daniela Curti, Francesca Botti, Gerardo Biella, Paolo Bigini, Tiziana Mennini & Mauro Toselli. (2012) Neural precursors (NPCs) from adult L967Q mice display early commitment to “in vitro” neuronal differentiation and hyperexcitability. Experimental Neurology 236:2, pages 307-318.
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Ralf Palmisano, Panagiota Golfi, Peter Heimann, Christopher Shaw, Claire Troakes, Thomas Schmitt-John & Jörg W Bartsch. (2011) Endosomal accumulation of APP in wobbler motor neurons reflects impaired vesicle trafficking: Implications for human motor neuron disease. BMC Neuroscience 12:1.
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L. Corrado, S. Gagliardi, Y. Carlomagno, T. Mennini, N. Ticozzi, L. Mazzini, V. Silani, C. Cereda & S. D’Alfonso. (2011) VPS54 genetic analysis in ALS Italian cohort. European Journal of Neurology 18:4, pages e41-e42.
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Juan S. Bonifacino & Aitor Hierro. (2011) Transport according to GARP: receiving retrograde cargo at the trans-Golgi network. Trends in Cell Biology 21:3, pages 159-167.
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Lisa Staunton, Harald Jockusch & Kay Ohlendieck. (2011) Proteomic analysis of muscle affected by motor neuron degeneration: The wobbler mouse model of amyotrophic lateral sclerosis. Biochemical and Biophysical Research Communications 406:4, pages 595-600.
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Eva Wohlleber, Maria Kirchhoff, Alexander M. Zink, Martina Kreiß-Nachtsheim, Alma Küchler, Birgit Jepsen, Susanne Kjaergaard & Hartmut Engels. (2011) Clinical and molecular characterization of two patients with overlapping de novo microdeletions in 2p14-p15 and mild mental retardation. European Journal of Medical Genetics 54:1, pages 67-72.
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Melissa NassifSoledad MatusKaren CastilloClaudio Hetz. (2010) Amyotrophic Lateral Sclerosis Pathogenesis: A Journey Through the Secretory Pathway. Antioxidants & Redox Signaling 13:12, pages 1955-1989.
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F. Javier Pérez-VictoriaGuillermo Abascal-PalaciosIgor TascónAndrey Kajava, Javier G. MagadánErik P. Pioro, Juan S. BonifacinoAitor Hierro. (2010) Structural basis for the wobbler mouse neurodegenerative disorder caused by mutation in the Vps54 subunit of the GARP complex. Proceedings of the National Academy of Sciences 107:29, pages 12860-12865.
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Antonio Bastone, Elena Fumagalli, Paolo Bigini, Pietro Perini, Davide Bernardinello, Alfredo Cagnotto, Ilario Mereghetti, Daniela Curti, Mario Salmona & Tiziana Mennini. (2009) Proteomic Profiling of Cervical and Lumbar Spinal Cord Reveals Potential Protective Mechanisms in the Wobbler Mouse, a Model of Motor Neuron Degeneration. Journal of Proteome Research 8:11, pages 5229-5240.
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J.S. Dennis & B.A. Citron. (2009) Wobbler mice modeling motor neuron disease display elevated transactive response DNA binding protein. Neuroscience 158:2, pages 745-750.
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