Thomas H. Gillingwater Centre for Integrative Physiology University of Edinburgh Biography Publications Institution JoVE Articles Thomas H. Gillingwater has not added a biography. If you are Thomas H. Gillingwater and would like to personalize this page please email our Author Liaison for assistance. Publications PTEN Depletion Decreases Disease Severity and Modestly Prolongs Survival in a Mouse Model of Spinal Muscular Atrophy Molecular Therapy : the Journal of the American Society of Gene Therapy. Nov, 2014 | Pubmed ID: 25369768 Loss of Glial Neurofascin155 Delays Developmental Synapse Elimination at the Neuromuscular Junction The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. Sep, 2014 | Pubmed ID: 25232125 Label-free Quantitative Proteomic Profiling Identifies Disruption of Ubiquitin Homeostasis As a Key Driver of Schwann Cell Defects in Spinal Muscular Atrophy Journal of Proteome Research. Nov, 2014 | Pubmed ID: 25151848 The Influence of Storage Parameters on Measurement of Survival Motor Neuron (SMN) Protein Levels: Implications for Pre-clinical Studies and Clinical Trials for Spinal Muscular Atrophy Neuromuscular Disorders : NMD. Jun, 2014 | Pubmed ID: 25047670 Increased Levels of UCHL1 Are a Compensatory Response to Disrupted Ubiquitin Homeostasis in Spinal Muscular Atrophy and Do Not Represent a Viable Therapeutic Target Neuropathology and Applied Neurobiology. Dec, 2014 | Pubmed ID: 25041530 A Novel Mouse Model of Warburg Micro Syndrome Reveals Roles for RAB18 in Eye Development and Organisation of the Neuronal Cytoskeleton Disease Models & Mechanisms. Apr, 2014 | Pubmed ID: 24764192 The Armadillo As a Model for Peripheral Neuropathy in Leprosy ILAR Journal / National Research Council, Institute of Laboratory Animal Resources. 2014 | Pubmed ID: 24615444 Dysregulation of Ubiquitin Homeostasis and β-catenin Signaling Promote Spinal Muscular Atrophy The Journal of Clinical Investigation. Apr, 2014 | Pubmed ID: 24590288 Morphological Analysis of Neuromuscular Junction Development and Degeneration in Rodent Lumbrical Muscles Journal of Neuroscience Methods. Apr, 2014 | Pubmed ID: 24530702 SMN-dependent Intrinsic Defects in Schwann Cells in Mouse Models of Spinal Muscular Atrophy Human Molecular Genetics. Dec, 2013 | Pubmed ID: 24301677 Label-free Proteomics Identifies Calreticulin and GRP75/Mortalin As Peripherally Accessible Protein Biomarkers for Spinal Muscular Atrophy Genome Medicine. Oct, 2013 | Pubmed ID: 24134804 Executive Deficits, Not Processing Speed Relates to Abnormalities in Distinct Prefrontal Tracts in Amyotrophic Lateral Sclerosis Brain : a Journal of Neurology. Nov, 2013 | Pubmed ID: 24056536 Total Protein Analysis As a Reliable Loading Control for Quantitative Fluorescent Western Blotting PloS One. 2013 | Pubmed ID: 24023619 Studying Synapses in Human Brain with Array Tomography and Electron Microscopy Nature Protocols. 2013 | Pubmed ID: 23787894 Increasing SMN Levels Using the Histone Deacetylase Inhibitor SAHA Ameliorates Defects in Skeletal Muscle Microvasculature in a Mouse Model of Severe Spinal Muscular Atrophy Neuroscience Letters. Jun, 2013 | Pubmed ID: 23583590 Effect of Limb Lengthening on Internodal Length and Conduction Velocity of Peripheral Nerve The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. Mar, 2013 | Pubmed ID: 23467369 Quantitative Tractography and Tract Shape Modeling in Amyotrophic Lateral Sclerosis Journal of Magnetic Resonance Imaging : JMRI. Nov, 2013 | Pubmed ID: 23450730 Spinal Muscular Atrophy: Going Beyond the Motor Neuron Trends in Molecular Medicine. Jan, 2013 | Pubmed ID: 23228902 Morphological Characteristics of Motor Neurons Do Not Determine Their Relative Susceptibility to Degeneration in a Mouse Model of Severe Spinal Muscular Atrophy PloS One. 2012 | Pubmed ID: 23285108 Combining Comparative Proteomics and Molecular Genetics Uncovers Regulators of Synaptic and Axonal Stability and Degeneration in Vivo PLoS Genetics. 2012 | Pubmed ID: 22952455 WldS Prevents Axon Degeneration Through Increased Mitochondrial Flux and Enhanced Mitochondrial Ca2+ Buffering Current Biology : CB. Apr, 2012 | Pubmed ID: 22425157 Altered Maturation of the Primary Somatosensory Cortex in a Mouse Model of Fragile X Syndrome Human Molecular Genetics. May, 2012 | Pubmed ID: 22328088 Development of a Supported Self-directed Learning Approach for Anatomy Education Anatomical Sciences Education. Mar-Apr, 2012 | Pubmed ID: 22223487 Using Induced Pluripotent Stem Cells (iPSC) to Model Human Neuromuscular Connectivity: Promise or Reality? Journal of Anatomy. Feb, 2012 | Pubmed ID: 22133357 Targeting Synaptic Pathology in Multiple Sclerosis: Fingolimod to the Rescue? British Journal of Pharmacology. Feb, 2012 | Pubmed ID: 21806598 Morphologic and Functional Correlates of Synaptic Pathology in the Cathepsin D Knockout Mouse Model of Congenital Neuronal Ceroid Lipofuscinosis Journal of Neuropathology and Experimental Neurology. Dec, 2011 | Pubmed ID: 22082660 Reversible Molecular Pathology of Skeletal Muscle in Spinal Muscular Atrophy Human Molecular Genetics. Nov, 2011 | Pubmed ID: 21840928 The Contribution of Mouse Models to Understanding the Pathogenesis of Spinal Muscular Atrophy Disease Models & Mechanisms. Jul, 2011 | Pubmed ID: 21708901 ApoE Isoform-specific Regulation of Regeneration in the Peripheral Nervous System Human Molecular Genetics. Jun, 2011 | Pubmed ID: 21478199 Induction of Cell Stress in Neurons from Transgenic Mice Expressing Yellow Fluorescent Protein: Implications for Neurodegeneration Research PloS One. 2011 | Pubmed ID: 21408118 The Response of Neuromuscular Junctions to Injury is Developmentally Regulated FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology. Apr, 2011 | Pubmed ID: 21228222 Synaptic Protection in the Brain of WldS Mice Occurs Independently of Age but is Sensitive to Gene-dose PloS One. 2010 | Pubmed ID: 21124744 SMN Deficiency Disrupts Brain Development in a Mouse Model of Severe Spinal Muscular Atrophy Human Molecular Genetics. Nov, 2010 | Pubmed ID: 20705736 Age-related Motor Neuron Degeneration in DNA Repair-deficient Ercc1 Mice Acta Neuropathologica. Oct, 2010 | Pubmed ID: 20602234 Pre-symptomatic Development of Lower Motor Neuron Connectivity in a Mouse Model of Severe Spinal Muscular Atrophy Human Molecular Genetics. Feb, 2010 | Pubmed ID: 19884170 Murine Cathepsin D Deficiency is Associated with Dysmyelination/myelin Disruption and Accumulation of Cholesteryl Esters in the Brain Journal of Neurochemistry. Jan, 2010 | Pubmed ID: 19845830 Alternative Splicing Events Are a Late Feature of Pathology in a Mouse Model of Spinal Muscular Atrophy PLoS Genetics. Dec, 2009 | Pubmed ID: 20019802 Expression of the Neuroprotective Slow Wallerian Degeneration (WldS) Gene in Non-neuronal Tissues BMC Neuroscience. 2009 | Pubmed ID: 20015399 Molecular Correlates of Axonal and Synaptic Pathology in Mouse Models of Batten Disease Human Molecular Genetics. Nov, 2009 | Pubmed ID: 19640925 Transcriptional Regulation of the AP-1 and Nrf2 Target Gene Sulfiredoxin Molecules and Cells. Mar, 2009 | Pubmed ID: 19326073 Protein Product of CLN6 Gene Responsible for Variant Late-onset Infantile Neuronal Ceroid Lipofuscinosis Interacts with CRMP-2 Journal of Neuroscience Research. Jul, 2009 | Pubmed ID: 19235893 The Importance of Exposure to Human Material in Anatomical Education: a Philosophical Perspective Anatomical Sciences Education. Nov-Dec, 2008 | Pubmed ID: 19109856 Loss of Translation Elongation Factor (eEF1A2) Expression in Vivo Differentiates Between Wallerian Degeneration and Dying-back Neuronal Pathology Journal of Anatomy. Dec, 2008 | Pubmed ID: 19094180 MGluR5 Regulates Glutamate-dependent Development of the Mouse Somatosensory Cortex The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. Dec, 2008 | Pubmed ID: 19052194 Identity, Developmental Restriction and Reactivity of Extralaminar Cells Capping Mammalian Neuromuscular Junctions Journal of Cell Science. Dec, 2008 | Pubmed ID: 19001504 Modified Cell Cycle Status in a Mouse Model of Altered Neuronal Vulnerability (slow Wallerian Degeneration; Wlds) Genome Biology. 2008 | Pubmed ID: 18570652 Rapid Loss of Motor Nerve Terminals Following Hypoxia-reperfusion Injury Occurs Via Mechanisms Distinct from Classic Wallerian Degeneration Journal of Anatomy. Jun, 2008 | Pubmed ID: 18510509 VCP Binding Influences Intracellular Distribution of the Slow Wallerian Degeneration Protein, Wld(S) Molecular and Cellular Neurosciences. Jul, 2008 | Pubmed ID: 18468455 VAPB Interacts with and Modulates the Activity of ATF6 Human Molecular Genetics. Jun, 2008 | Pubmed ID: 18263603 A Neurological Phenotype in Mice with DNA Repair Gene Ercc1 Deficiency DNA Repair. Feb, 2008 | Pubmed ID: 18221731 Synaptic Changes in the Thalamocortical System of Cathepsin D-deficient Mice: a Model of Human Congenital Neuronal Ceroid-lipofuscinosis Journal of Neuropathology and Experimental Neurology. Jan, 2008 | Pubmed ID: 18091563 Selective Vulnerability of Motor Neurons and Dissociation of Pre- and Post-synaptic Pathology at the Neuromuscular Junction in Mouse Models of Spinal Muscular Atrophy Human Molecular Genetics. Apr, 2008 | Pubmed ID: 18065780 Design of a Novel Quantitative PCR (QPCR)-based Protocol for Genotyping Mice Carrying the Neuroprotective Wallerian Degeneration Slow (Wlds) Gene Molecular Neurodegeneration. 2007 | Pubmed ID: 17971231 Differential Proteomics Analysis of Synaptic Proteins Identifies Potential Cellular Targets and Protein Mediators of Synaptic Neuroprotection Conferred by the Slow Wallerian Degeneration (Wlds) Gene Molecular & Cellular Proteomics : MCP. Aug, 2007 | Pubmed ID: 17470424 Synaptic Vulnerability in Neurodegenerative Disease Journal of Neuropathology and Experimental Neurology. Aug, 2006 | Pubmed ID: 16896307 Delayed Synaptic Degeneration in the CNS of Wlds Mice After Cortical Lesion Brain : a Journal of Neurology. Jun, 2006 | Pubmed ID: 16738060 Involvement of Protein Kinase A in Patterning of the Mouse Somatosensory Cortex The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. May, 2006 | Pubmed ID: 16707791 Synaptic Ras GTPase Activating Protein Regulates Pattern Formation in the Trigeminal System of Mice The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. Feb, 2006 | Pubmed ID: 16452659 The Neuroprotective WldS Gene Regulates Expression of PTTG1 and Erythroid Differentiation Regulator 1-like Gene in Mice and Human Cells Human Molecular Genetics. Feb, 2006 | Pubmed ID: 16403805 Progressive Loss of Motor Neuron Function in Wasted Mice: Effects of a Spontaneous Null Mutation in the Gene for the EEF1 A2 Translation Factor Journal of Neuropathology and Experimental Neurology. Apr, 2005 | Pubmed ID: 15835265 A Rat Model of Slow Wallerian Degeneration (WldS) with Improved Preservation of Neuromuscular Synapses The European Journal of Neuroscience. Jan, 2005 | Pubmed ID: 15654865 The Slow Wallerian Degeneration Gene, WldS, Inhibits Axonal Spheroid Pathology in Gracile Axonal Dystrophy Mice Brain : a Journal of Neurology. Feb, 2005 | Pubmed ID: 15644421 Progressive Abnormalities in Skeletal Muscle and Neuromuscular Junctions of Transgenic Mice Expressing the Huntington's Disease Mutation The European Journal of Neuroscience. Dec, 2004 | Pubmed ID: 15579164 Myo-GDNF Increases Non-functional Polyinnervation of Reinnervated Mouse Muscle Neuroreport. Jan, 2004 | Pubmed ID: 15106825 Axotomy-dependent and -independent Synapse Elimination in Organ Cultures of Wld(s) Mutant Mouse Skeletal Muscle Journal of Neuroscience Research. Apr, 2004 | Pubmed ID: 15048930 Neuroprotection After Transient Global Cerebral Ischemia in Wld(s) Mutant Mice Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism. Jan, 2004 | Pubmed ID: 14688617 The Relationship of Neuromuscular Synapse Elimination to Synaptic Degeneration and Pathology: Insights from WldS and Other Mutant Mice Journal of Neurocytology. Jun-Sep, 2003 | Pubmed ID: 15034273 Ultrastructural Correlates of Synapse Withdrawal at Axotomized Neuromuscular Junctions in Mutant and Transgenic Mice Expressing the Wld Gene Journal of Anatomy. Sep, 2003 | Pubmed ID: 14529044 Age-dependent Synapse Withdrawal at Axotomised Neuromuscular Junctions in Wld(s) Mutant and Ube4b/Nmnat Transgenic Mice The Journal of Physiology. Sep, 2002 | Pubmed ID: 12231635 A Guide to Modern Quantitative Fluorescent Western Blotting with Troubleshooting Strategies Samantha L. Eaton1, Maica Llavero Hurtado1, Karla J. Oldknow2, Laura C. Graham1, Thomas W. Marchant1, Thomas H. Gillingwater3,4, Colin Farquharson2, Thomas M. Wishart1,4 1Division of Neurobiology, The Roslin Institute and Royal (Dick) School of Veterinary Studies, University of Edinburgh, 2Division of Developmental Biology, The Roslin Institute and Royal (Dick) School of Veterinary Studies, University of Edinburgh, 3Centre for Integrative Physiology, University of Edinburgh, 4Euan MacDonald Centre for Motor Neurone Disease Research, University of Edinburgh JoVE 52099 Biologia
A Guide to Modern Quantitative Fluorescent Western Blotting with Troubleshooting Strategies Samantha L. Eaton1, Maica Llavero Hurtado1, Karla J. Oldknow2, Laura C. Graham1, Thomas W. Marchant1, Thomas H. Gillingwater3,4, Colin Farquharson2, Thomas M. Wishart1,4 1Division of Neurobiology, The Roslin Institute and Royal (Dick) School of Veterinary Studies, University of Edinburgh, 2Division of Developmental Biology, The Roslin Institute and Royal (Dick) School of Veterinary Studies, University of Edinburgh, 3Centre for Integrative Physiology, University of Edinburgh, 4Euan MacDonald Centre for Motor Neurone Disease Research, University of Edinburgh JoVE 52099 Biologia