Axonal Transport Defects in a Mitofusin 2 Loss of Function Model of Charcot-Marie-Tooth Disease in Zebrafish

Autor: Chapman, AL, Bennett, EJ, Ramesh, TM, De Vos, KJ, Grierson, AJ

Publikováno v: PLoS ONE, Vol 8, Iss 6, p e67276 (2013)
PLoS ONE

Charcot-Marie-Tooth disease (CMT) represents a group of neurodegenerative disorders typically characterised by demyelination (CMT1) or distal axon degeneration (CMT2) of motor and sensory neurons. The majority of CMT2 cases are caused by mutations in

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid_dedup__::d11f1043b04e0340dbc341c16dfbdd59
https://doaj.org/article/ab40266d2dcc4698a9f62aba426eedbf

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Loss of TMEM106B exacerbates C9ALS/FTD DPR pathology by disrupting autophagosome maturation.

Autor: Bauer CS; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, United Kingdom.; Neuroscience Institute, University of Sheffield, Sheffield, United Kingdom., Webster CP; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, United Kingdom.; Neuroscience Institute, University of Sheffield, Sheffield, United Kingdom., Shaw AC; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, United Kingdom.; Neuroscience Institute, University of Sheffield, Sheffield, United Kingdom., Kok JR; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, United Kingdom.; Neuroscience Institute, University of Sheffield, Sheffield, United Kingdom., Castelli LM; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, United Kingdom.; Neuroscience Institute, University of Sheffield, Sheffield, United Kingdom., Lin YH; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, United Kingdom.; Neuroscience Institute, University of Sheffield, Sheffield, United Kingdom., Smith EF; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, United Kingdom.; Neuroscience Institute, University of Sheffield, Sheffield, United Kingdom., Illanes-Álvarez F; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, United Kingdom.; Neuroscience Institute, University of Sheffield, Sheffield, United Kingdom., Higginbottom A; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, United Kingdom.; Neuroscience Institute, University of Sheffield, Sheffield, United Kingdom., Shaw PJ; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, United Kingdom.; Neuroscience Institute, University of Sheffield, Sheffield, United Kingdom., Azzouz M; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, United Kingdom.; Neuroscience Institute, University of Sheffield, Sheffield, United Kingdom., Ferraiuolo L; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, United Kingdom.; Neuroscience Institute, University of Sheffield, Sheffield, United Kingdom., Hautbergue GM; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, United Kingdom.; Neuroscience Institute, University of Sheffield, Sheffield, United Kingdom., Grierson AJ; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, United Kingdom.; Neuroscience Institute, University of Sheffield, Sheffield, United Kingdom., De Vos KJ; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, United Kingdom.; Neuroscience Institute, University of Sheffield, Sheffield, United Kingdom.

Publikováno v: Frontiers in cellular neuroscience [Front Cell Neurosci] 2022 Dec 16; Vol. 16, pp. 1061559. Date of Electronic Publication: 2022 Dec 16 (Print Publication: 2022).

C9ORF72 -derived poly-GA DPRs undergo endocytic uptake in iAstrocytes and spread to motor neurons.

Autor: Marchi PM; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, UK.; Neuroscience Institute, University of Sheffield, Western Bank, Sheffield, UK., Marrone L; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, UK.; Neuroscience Institute, University of Sheffield, Western Bank, Sheffield, UK., Brasseur L; The French Alternative Energies and Atomic Energy Commission (CEA), Institut François Jacob (MIRcen) and The French National Centre for Scientific Research (CNRS), Laboratory of Neurodegenerative Diseases (UMR9199), Fontenay-aux-Roses, France., Coens A; The French Alternative Energies and Atomic Energy Commission (CEA), Institut François Jacob (MIRcen) and The French National Centre for Scientific Research (CNRS), Laboratory of Neurodegenerative Diseases (UMR9199), Fontenay-aux-Roses, France., Webster CP; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, UK.; Neuroscience Institute, University of Sheffield, Western Bank, Sheffield, UK., Bousset L; The French Alternative Energies and Atomic Energy Commission (CEA), Institut François Jacob (MIRcen) and The French National Centre for Scientific Research (CNRS), Laboratory of Neurodegenerative Diseases (UMR9199), Fontenay-aux-Roses, France., Destro M; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, UK.; Neuroscience Institute, University of Sheffield, Western Bank, Sheffield, UK., Smith EF; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, UK.; Neuroscience Institute, University of Sheffield, Western Bank, Sheffield, UK.; Centre for Membrane Interactions and Dynamics, University of Sheffield, Western Bank, Sheffield, UK., Walther CG; The Wolfson Light Microscopy Facility, University of Sheffield, Sheffield, UK., Alfred V; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, UK.; Neuroscience Institute, University of Sheffield, Western Bank, Sheffield, UK., Marroccella R; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, UK.; Neuroscience Institute, University of Sheffield, Western Bank, Sheffield, UK., Graves EJ; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, UK.; Neuroscience Institute, University of Sheffield, Western Bank, Sheffield, UK., Robinson D; The Wolfson Light Microscopy Facility, University of Sheffield, Sheffield, UK., Shaw AC; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, UK.; Neuroscience Institute, University of Sheffield, Western Bank, Sheffield, UK., Wan LM; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, UK.; Neuroscience Institute, University of Sheffield, Western Bank, Sheffield, UK., Grierson AJ; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, UK.; Neuroscience Institute, University of Sheffield, Western Bank, Sheffield, UK., Ebbens SJ; Department of Chemical and Biological Engineering, University of Sheffield, Sheffield, UK., De Vos KJ; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, UK.; Neuroscience Institute, University of Sheffield, Western Bank, Sheffield, UK.; Centre for Membrane Interactions and Dynamics, University of Sheffield, Western Bank, Sheffield, UK., Hautbergue GM; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, UK.; Neuroscience Institute, University of Sheffield, Western Bank, Sheffield, UK., Ferraiuolo L; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, UK.; Neuroscience Institute, University of Sheffield, Western Bank, Sheffield, UK., Melki R; The French Alternative Energies and Atomic Energy Commission (CEA), Institut François Jacob (MIRcen) and The French National Centre for Scientific Research (CNRS), Laboratory of Neurodegenerative Diseases (UMR9199), Fontenay-aux-Roses, France ronald.melki@cnrs.fr m.azzouz@sheffield.ac.uk., Azzouz M; Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, UK ronald.melki@cnrs.fr m.azzouz@sheffield.ac.uk.; Neuroscience Institute, University of Sheffield, Western Bank, Sheffield, UK.

Publikováno v: Life science alliance [Life Sci Alliance] 2022 May 13; Vol. 5 (9). Date of Electronic Publication: 2022 May 13 (Print Publication: 2022).