Centre for integrative Physiology,
Euan MacDonald Centre for Motor Neurone Disease Research,
Centre for Discovery Brain Sciences
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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
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
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
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
Myo-GDNF increases non-functional polyinnervation of reinnervated mouse muscle.
Neuroreport Jan, 2004 | Pubmed ID: 15106825
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
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
A rat model of slow Wallerian degeneration (WldS) with improved preservation of neuromuscular synapses.
The European journal of neuroscience Jan, 2005 | Pubmed ID: 15654865
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
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
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
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
Delayed synaptic degeneration in the CNS of Wlds mice after cortical lesion.
Brain : a journal of neurology Jun, 2006 | Pubmed ID: 16738060
Synaptic vulnerability in neurodegenerative disease.
Journal of neuropathology and experimental neurology Aug, 2006 | Pubmed ID: 16896307
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
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
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
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
A neurological phenotype in mice with DNA repair gene Ercc1 deficiency.
DNA repair Feb, 2008 | Pubmed ID: 18221731
VAPB interacts with and modulates the activity of ATF6.
Human molecular genetics Jun, 2008 | Pubmed ID: 18263603
VCP binding influences intracellular distribution of the slow Wallerian degeneration protein, Wld(S).
Molecular and cellular neurosciences Jul, 2008 | Pubmed ID: 18468455
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
Modified cell cycle status in a mouse model of altered neuronal vulnerability (slow Wallerian degeneration; Wlds).
Genome biology , 2008 | Pubmed ID: 18570652
Identity, developmental restriction and reactivity of extralaminar cells capping mammalian neuromuscular junctions.
Journal of cell science Dec, 2008 | Pubmed ID: 19001504
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
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
The importance of exposure to human material in anatomical education: a philosophical perspective.
Anatomical sciences education Nov-Dec, 2008 | Pubmed ID: 19109856
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
Transcriptional regulation of the AP-1 and Nrf2 target gene sulfiredoxin.
Molecules and cells Mar, 2009 | Pubmed ID: 19326073
Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease.
Human molecular genetics Nov, 2009 | Pubmed ID: 19640925
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
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
Expression of the neuroprotective slow Wallerian degeneration (WldS) gene in non-neuronal tissues.
BMC neuroscience , 2009 | Pubmed ID: 20015399
Alternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophy.
PLoS genetics Dec, 2009 | Pubmed ID: 20019802
Age-related motor neuron degeneration in DNA repair-deficient Ercc1 mice.
Acta neuropathologica Oct, 2010 | Pubmed ID: 20602234
SMN deficiency disrupts brain development in a mouse model of severe spinal muscular atrophy.
Human molecular genetics Nov, 2010 | Pubmed ID: 20705736
Synaptic protection in the brain of WldS mice occurs independently of age but is sensitive to gene-dose.
PloS one , 2010 | Pubmed ID: 21124744
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
Induction of cell stress in neurons from transgenic mice expressing yellow fluorescent protein: implications for neurodegeneration research.
PloS one , 2011 | Pubmed ID: 21408118
ApoE isoform-specific regulation of regeneration in the peripheral nervous system.
Human molecular genetics Jun, 2011 | Pubmed ID: 21478199
The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy.
Disease models & mechanisms Jul, 2011 | Pubmed ID: 21708901
Targeting synaptic pathology in multiple sclerosis: fingolimod to the rescue?
British journal of pharmacology Feb, 2012 | Pubmed ID: 21806598
Reversible molecular pathology of skeletal muscle in spinal muscular atrophy.
Human molecular genetics Nov, 2011 | Pubmed ID: 21840928
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
Using induced pluripotent stem cells (iPSC) to model human neuromuscular connectivity: promise or reality?
Journal of anatomy Feb, 2012 | Pubmed ID: 22133357
Development of a supported self-directed learning approach for anatomy education.
Anatomical sciences education Mar-Apr, 2012 | Pubmed ID: 22223487
Altered maturation of the primary somatosensory cortex in a mouse model of fragile X syndrome.
Human molecular genetics May, 2012 | Pubmed ID: 22328088
WldS prevents axon degeneration through increased mitochondrial flux and enhanced mitochondrial Ca2+ buffering.
Current biology : CB Apr, 2012 | Pubmed ID: 22425157
Combining comparative proteomics and molecular genetics uncovers regulators of synaptic and axonal stability and degeneration in vivo.
PLoS genetics , 2012 | Pubmed ID: 22952455
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
Quantitative tractography and tract shape modeling in amyotrophic lateral sclerosis.
Journal of magnetic resonance imaging : JMRI Nov, 2013 | Pubmed ID: 23450730
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
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
Studying synapses in human brain with array tomography and electron microscopy.
Nature protocols , 2013 | Pubmed ID: 23787894
Total protein analysis as a reliable loading control for quantitative fluorescent Western blotting.
PloS one , 2013 | Pubmed ID: 24023619
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
Label-free proteomics identifies Calreticulin and GRP75/Mortalin as peripherally accessible protein biomarkers for spinal muscular atrophy.
Genome medicine Oct, 2013 | Pubmed ID: 24134804
SMN-dependent intrinsic defects in Schwann cells in mouse models of spinal muscular atrophy.
Human molecular genetics Dec, 2013 | Pubmed ID: 24301677
Morphological analysis of neuromuscular junction development and degeneration in rodent lumbrical muscles.
Journal of neuroscience methods Apr, 2014 | Pubmed ID: 24530702
Dysregulation of ubiquitin homeostasis and β-catenin signaling promote spinal muscular atrophy.
The Journal of clinical investigation Apr, 2014 | Pubmed ID: 24590288
The armadillo as a model for peripheral neuropathy in leprosy.
ILAR journal / National Research Council, Institute of Laboratory Animal Resources , 2014 | Pubmed ID: 24615444
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
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
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
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
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
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
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