James N. Sleigh Department of Neuromuscular Diseases UCL Queen Square Institute of Neurology Biography Publications Institution JoVE Articles James N. Sleigh James N. Sleigh received his undergraduate Masters in Biology (MBiol) from the University of Bath (2005-2009), which included a year at Harvard Medical School (2007-2008) researching the neuromuscular disease spinal muscular atrophy (SMA). Supported by the UK Medical Research Council (MRC), James then completed his DPhil at the University of Oxford (2009-2012), extending his work on SMA, while studying a number of other disorders impacting peripheral motor and sensory nerves. From 2012-2014, James worked in the laboratory of Dr. Zameel Cader on the genetic peripheral neuropathy Charcot-Marie-Tooth disease (CMT). During a four year, Wellcome Trust-funded Sir Henry Wellcome Postdoctoral Fellowship (2014-2018), he continued his research on genetic peripheral neuropathy at the UCL Queen Square Institute of Neurology working with Prof. Giampietro Schiavo.Funded by a Career Development Award (2019-2024) from the MRC, James is now a Senior Research Fellow in the Department of Neuromuscular Diseases, leading a team working on causes of motor and sensory nerve degeneration and the mechanisms underpinning the dynamic cellular process of axonal transport. By improving understanding of neuropathic pathways and associated pathologies, the Sleigh Laboratory aims to generate pre-clinical molecular therapies for genetic peripheral nerve diseases. Publications Dissection, in Vivo Imaging and Analysis of the Mouse Epitrochleoanconeus Muscle Journal of Anatomy. Jun, 2021 | Pubmed ID: 34121181 NMJ-Analyser Identifies Subtle Early Changes in Mouse Models of Neuromuscular Disease Scientific Reports. 06, 2021 | Pubmed ID: 34112844 Altered Sensory Neuron Development in CMT2D Mice Is Site-Specific and Linked to Increased GlyRS Levels Frontiers in Cellular Neuroscience. Month, 2020 | Pubmed ID: 32848623 Intramuscular Delivery of Gene Therapy for Targeting the Nervous System Frontiers in Molecular Neuroscience. Month, 2020 | Pubmed ID: 32765219 Developmental Demands Contribute to Early Neuromuscular Degeneration in CMT2D Mice Cell Death & Disease. 07, 2020 | Pubmed ID: 32703932 A Video Protocol for Rapid Dissection of Mouse Dorsal Root Ganglia from Defined Spinal Levels BMC Research Notes. Jun, 2020 | Pubmed ID: 32580748 Morphological Variability is Greater at Developing Than Mature Mouse Neuromuscular Junctions Journal of Anatomy. Jun, 2020 | Pubmed ID: 32533580 In Vivo Imaging of Anterograde and Retrograde Axonal Transport in Rodent Peripheral Nerves Methods in Molecular Biology (Clifton, N.J.). Month, 2020 | Pubmed ID: 32524487 Mice Carrying ALS Mutant TDP-43, but Not Mutant FUS, Display In Vivo Defects in Axonal Transport of Signaling Endosomes Cell Reports. Mar, 2020 | Pubmed ID: 32187538 Loss of BICD2 in Muscle Drives Motor Neuron Loss in a Developmental Form of Spinal Muscular Atrophy Acta Neuropathologica Communications. 03, 2020 | Pubmed ID: 32183910 The Evolution of the Axonal Transport Toolkit Traffic (Copenhagen, Denmark). 01, 2020 | Pubmed ID: 31670447 Neuronal Over-expression of Oxr1 is Protective Against ALS-associated Mutant TDP-43 Mislocalisation in Motor Neurons and Neuromuscular Defects in Vivo Human Molecular Genetics. 11, 2019 | Pubmed ID: 31642482 Axonal Transport and Neurological Disease Nature Reviews. Neurology. 12, 2019 | Pubmed ID: 31558780 UBA1/GARS-dependent Pathways Drive Sensory-motor Connectivity Defects in Spinal Muscular Atrophy Brain : a Journal of Neurology. Oct, 2018 | Pubmed ID: 30239612 Aligned Electrospun Fibers for Neural Patterning Biotechnology Letters. Mar, 2018 | Pubmed ID: 29313254 Motor Neuron Gene Therapy: Lessons from Spinal Muscular Atrophy for Amyotrophic Lateral Sclerosis Frontiers in Molecular Neuroscience. Month, 2017 | Pubmed ID: 29270111 Neuropilin 1 Sequestration by Neuropathogenic Mutant Glycyl-tRNA Synthetase is Permissive to Vascular Homeostasis Scientific Reports. 08, 2017 | Pubmed ID: 28835631 Trk Receptor Signaling and Sensory Neuron Fate Are Perturbed in Human Neuropathy Caused by Mutations Proceedings of the National Academy of Sciences of the United States of America. 04, 2017 | Pubmed ID: 28351971 Methodological Advances in Imaging Intravital Axonal Transport F1000Research. Month, 2017 | Pubmed ID: 28344778 Systemic Peptide-mediated Oligonucleotide Therapy Improves Long-term Survival in Spinal Muscular Atrophy Proceedings of the National Academy of Sciences of the United States of America. 09, 2016 | Pubmed ID: 27621445 A Simple, Step-by-step Dissection Protocol for the Rapid Isolation of Mouse Dorsal Root Ganglia BMC Research Notes. Feb, 2016 | Pubmed ID: 26864470 Vascular Defects and Spinal Cord Hypoxia in Spinal Muscular Atrophy Annals of Neurology. Feb, 2016 | Pubmed ID: 26506088 In Vivo Imaging of Axonal Transport in Murine Motor and Sensory Neurons Journal of Neuroscience Methods. Jan, 2016 | Pubmed ID: 26424507 Deacetylation of Miro1 by HDAC6 Blocks Mitochondrial Transport and Mediates Axon Growth Inhibition The Journal of Cell Biology. Month, | Pubmed ID: 31068376 Plexin-Semaphorin Signaling Modifies Neuromuscular Defects in a Model of Peripheral Neuropathy Frontiers in Molecular Neuroscience. Month, | Pubmed ID: 29520219 Antisense Oligonucleotides and Other Genetic Therapies Made Simple Practical Neurology. Month, | Pubmed ID: 29455156 Expanding the Toolkit for In Vivo Imaging of Axonal Transport Andrew P. Tosolini1,2, David Villarroel-Campos1,2,3, Giampietro Schiavo1,2,3, James N. Sleigh1,2,3 1Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, University College London, 2UCL Queen Square Motor Neuron Disease Centre, University College London, 3UK Dementia Research Institute, University College London JoVE 63471 Neurosciences
Expanding the Toolkit for In Vivo Imaging of Axonal Transport Andrew P. Tosolini1,2, David Villarroel-Campos1,2,3, Giampietro Schiavo1,2,3, James N. Sleigh1,2,3 1Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, University College London, 2UCL Queen Square Motor Neuron Disease Centre, University College London, 3UK Dementia Research Institute, University College London JoVE 63471 Neurosciences