Division of Neurobiology,
The Roslin Institute and Royal (Dick) School of Veterinary Studies,
Euan MacDonald Centre for Motor Neurone Disease Research,
Division of Neurobiology, The Roslin Institute and Royal (Dick) School of Veterinary Studies
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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 protein, WldS, binds directly to VCP/p97 and partially redistributes it within the nucleus.
Molecular biology of the cell Mar, 2006 | Pubmed ID: 16371511
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
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
VCP binding influences intracellular distribution of the slow Wallerian degeneration protein, Wld(S).
Molecular and cellular neurosciences Jul, 2008 | Pubmed ID: 18468455
Modified cell cycle status in a mouse model of altered neuronal vulnerability (slow Wallerian degeneration; Wlds).
Genome biology , 2008 | Pubmed ID: 18570652
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
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
Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease.
Human molecular genetics Nov, 2009 | Pubmed ID: 19640925
Expression of the neuroprotective slow Wallerian degeneration (WldS) gene in non-neuronal tissues.
BMC neuroscience , 2009 | Pubmed ID: 20015399
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
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
Reversible molecular pathology of skeletal muscle in spinal muscular atrophy.
Human molecular genetics Nov, 2011 | Pubmed ID: 21840928
Using induced pluripotent stem cells (iPSC) to model human neuromuscular connectivity: promise or reality?
Journal of anatomy Feb, 2012 | Pubmed ID: 22133357
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
Total protein analysis as a reliable loading control for quantitative fluorescent Western blotting.
PloS one , 2013 | Pubmed ID: 24023619
Label-free proteomics identifies Calreticulin and GRP75/Mortalin as peripherally accessible protein biomarkers for spinal muscular atrophy.
Genome medicine Oct, 2013 | Pubmed ID: 24134804
Dysregulation of ubiquitin homeostasis and β-catenin signaling promote spinal muscular atrophy.
The Journal of clinical investigation Apr, 2014 | Pubmed ID: 24590288
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 rat striatum responds to nigro-striatal degeneration via the increased expression of proteins associated with growth and regeneration of neuronal circuitry.
Proteome science , 2014 | Pubmed ID: 24834013
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
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
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
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