Department of Neuromuscular Diseases,
UCL Queen Square Institute of Neurology and UK Dementia Research Institute at UCL,
Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology and UK Dementia Research Institute at UCL
Professor Giampietro (Gipi) Schiavo is Deputy Director of the Department of Neuromuscular Diseases at University College London (UCL) Queen Square Institute of Neurology and head of the Molecular Neuropathobiology Laboratory at UCL. He is a UK Dementia Research Institute Investigator and the academic lead of the Alzheimer’s Research UK Drug Discovery Institute at UCL.
Professor Schiavo studied with Professor Cesare Montecucco at the University of Padova (Italy) and with Professor James Rothman at the Memorial Sloan Kettering Cancer Center in New York. He has a long-term interest in the mechanisms of action of bacterial protein toxins, in particular tetanus and botulinum neurotoxins, and their exploitation as tools in cell biology. Using these probes, his group has clarified key steps in the mechanism of ligand entry at the neuromuscular junction and other synapses and the recruitment of ligand-receptor complexes to signaling endosomes moving along the axonal retrograde transport route. This essential transport pathway, which delivers a variety of organelles and molecular complexes to the cell body of neurons, is impaired in several nervous system pathologies, such as motor neuron disease, Alzheimer’s disease, and Charcot-Marie-Tooth disease.
Professor Schiavo and his team are identifying novel pharmacological nodes to restore axonal transport of key organelles such as signaling endosomes, mitochondria, and lysosomes in neurons both in vitro and in vivo.
The journey of tetanus and botulinum neurotoxins in neurons.
Trends in microbiology Sep, 2003 | Pubmed ID: 13678859
Phosphatidylinositol 3-kinase C2alpha is essential for ATP-dependent priming of neurosecretory granule exocytosis.
Molecular biology of the cell Oct, 2005 | Pubmed ID: 16055506
Large-scale pathways-based association study in amyotrophic lateral sclerosis.
Brain : a journal of neurology Sep, 2007 | Pubmed ID: 17439985
Deficits in axonal transport precede ALS symptoms in vivo.
Proceedings of the National Academy of Sciences of the United States of America Nov, 2010 | Pubmed ID: 21059924
A motor-driven mechanism for cell-length sensing.
Cell reports Jun, 2012 | Pubmed ID: 22773964
Alternative fates of newly formed PrPSc upon prion conversion on the plasma membrane.
Journal of cell science Aug, 2013 | Pubmed ID: 23813960
Synthetic self-assembling clostridial chimera for modulation of sensory functions.
Bioconjugate chemistry Oct, 2013 | Pubmed ID: 24011174
Cytoplasmic dynein heavy chain: the servant of many masters.
Trends in neurosciences Nov, 2013 | Pubmed ID: 24035135
Signalling endosomes in axonal transport: travel updates on the molecular highway.
Seminars in cell & developmental biology Mar, 2014 | Pubmed ID: 24171925
TiME for TMEM106B.
The EMBO journal Mar, 2014 | Pubmed ID: 24497553
Rabies virus envelope glycoprotein targets lentiviral vectors to the axonal retrograde pathway in motor neurons.
The Journal of biological chemistry Jun, 2014 | Pubmed ID: 24753246
DYNC1H1 mutation alters transport kinetics and ERK1/2-cFos signalling in a mouse model of distal spinal muscular atrophy.
Brain : a journal of neurology Jul, 2014 | Pubmed ID: 24755273
siRNA screen of ES cell-derived motor neurons identifies novel regulators of tetanus toxin and neurotrophin receptor trafficking.
Frontiers in cellular neuroscience , 2014 | Pubmed ID: 24904285
Bicaudal-D1 regulates the intracellular sorting and signalling of neurotrophin receptors.
The EMBO journal Jul, 2014 | Pubmed ID: 24920579
Dysregulation of gene expression as a cause of Cockayne syndrome neurological disease.
Proceedings of the National Academy of Sciences of the United States of America Oct, 2014 | Pubmed ID: 25249633
Tetanus toxin entry. Nidogens are therapeutic targets for the prevention of tetanus.
Science (New York, N.Y.) Nov, 2014 | Pubmed ID: 25430769
Novel Kidins220/ARMS Splice Isoforms: Potential Specific Regulators of Neuronal and Cardiovascular Development.
PloS one , 2015 | Pubmed ID: 26083449
Evaluation of helper-dependent canine adenovirus vectors in a 3D human CNS model.
Gene therapy Jan, 2016 | Pubmed ID: 26181626
Regulation of Axonal Transport by Protein Kinases.
Trends in biochemical sciences Oct, 2015 | Pubmed ID: 26410600
In vivo imaging of axonal transport in murine motor and sensory neurons.
Journal of neuroscience methods Jan, 2016 | Pubmed ID: 26424507
Mon1-Ccz1 activates Rab7 only on late endosomes and dissociates from the lysosome in mammalian cells.
Journal of cell science Jan, 2016 | Pubmed ID: 26627821
Analysis of Signaling Endosome Composition and Dynamics Using SILAC in Embryonic Stem Cell-Derived Neurons.
Molecular & cellular proteomics : MCP Feb, 2016 | Pubmed ID: 26685126
A simple, step-by-step dissection protocol for the rapid isolation of mouse dorsal root ganglia.
BMC research notes Feb, 2016 | Pubmed ID: 26864470
Targeting protein homeostasis in sporadic inclusion body myositis.
Science translational medicine Mar, 2016 | Pubmed ID: 27009270
The Dynamic Localization of Cytoplasmic Dynein in Neurons Is Driven by Kinesin-1.
Neuron 06, 2016 | Pubmed ID: 27210554
Coxsackievirus Adenovirus Receptor Loss Impairs Adult Neurogenesis, Synapse Content, and Hippocampus Plasticity.
The Journal of neuroscience : the official journal of the Society for Neuroscience 09, 2016 | Pubmed ID: 27629708
Neuronal retrograde transport of Borna disease virus occurs in signalling endosomes.
The Journal of general virology Dec, 2016 | Pubmed ID: 27902378
Comparative analyses of glycerotoxin expression unveil a novel structural organization of the bloodworm venom system.
BMC evolutionary biology 03, 2017 | Pubmed ID: 28259138
Methodological advances in imaging intravital axonal transport.
F1000Research , 2017 | Pubmed ID: 28344778
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
Neuropilin 1 sequestration by neuropathogenic mutant glycyl-tRNA synthetase is permissive to vascular homeostasis.
Scientific reports 08, 2017 | Pubmed ID: 28835631
The travel diaries of tetanus and botulinum neurotoxins.
Toxicon : official journal of the International Society on Toxinology Jun, 2018 | Pubmed ID: 29031941
A neuroprotective astrocyte state is induced by neuronal signal EphB1 but fails in ALS models.
Nature communications 10, 2017 | Pubmed ID: 29079839
Compartmentalized Signaling in Neurons: From Cell Biology to Neuroscience.
Neuron Nov, 2017 | Pubmed ID: 29096079
Functional imaging in microfluidic chambers reveals sensory neuron sensitivity is differentially regulated between neuronal regions.
Pain Jul, 2018 | Pubmed ID: 29419650
Inhibiting p38 MAPK alpha rescues axonal retrograde transport defects in a mouse model of ALS.
Cell death & disease 05, 2018 | Pubmed ID: 29789529
The many disguises of the signalling endosome.
FEBS letters 11, 2018 | Pubmed ID: 30176054
UBA1/GARS-dependent pathways drive sensory-motor connectivity defects in spinal muscular atrophy.
Brain : a journal of neurology Oct, 2018 | Pubmed ID: 30239612
Retrograde transport of Akt by a neuronal Rab5-APPL1 endosome.
Scientific reports 02, 2019 | Pubmed ID: 30792402
Toxins 2017 conference in Madrid.
Toxicon : official journal of the International Society on Toxinology Jun, 2018 | Pubmed ID: 30837060
Spatiotemporal Control of ULK1 Activation by NDP52 and TBK1 during Selective Autophagy.
Molecular cell 04, 2019 | Pubmed ID: 30853401
Deacetylation of Miro1 by HDAC6 blocks mitochondrial transport and mediates axon growth inhibition.
The Journal of cell biology , | Pubmed ID: 31068376
Differential regulation of Kidins220 isoforms in Huntington's disease.
Brain pathology (Zurich, Switzerland) 01, 2020 | Pubmed ID: 31264746
Axonal transport and neurological disease.
Nature reviews. Neurology 12, 2019 | Pubmed ID: 31558780
TSPAN5 Enriched Microdomains Provide a Platform for Dendritic Spine Maturation through Neuroligin-1 Clustering.
Cell reports 10, 2019 | Pubmed ID: 31665629
The evolution of the axonal transport toolkit.
Traffic (Copenhagen, Denmark) 01, 2020 | Pubmed ID: 31670447
Travelling Together: A Unifying Pathomechanism for ALS.
Trends in neurosciences Jan, 2020 | Pubmed ID: 31744630
IGF1R regulates retrograde axonal transport of signalling endosomes in motor neurons.
EMBO reports Mar, 2020 | Pubmed ID: 32030864
PTPN23 binds the dynein adaptor BICD1 and is required for endocytic sorting of neurotrophin receptors.
Journal of cell science Mar, 2020 | Pubmed ID: 32079660
DYNLRB1 is essential for dynein mediated transport and neuronal survival.
Neurobiology of disease Jul, 2020 | Pubmed ID: 32088381
Duplication of clostridial binding domains for enhanced macromolecular delivery into neurons.
Toxicon: X Mar, 2020 | Pubmed ID: 32140681
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
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
FUS ALS-causative mutations impair FUS autoregulation and splicing factor networks through intron retention.
Nucleic acids research Jul, 2020 | Pubmed ID: 32479602
In Vivo Imaging of Anterograde and Retrograde Axonal Transport in Rodent Peripheral Nerves.
Methods in molecular biology (Clifton, N.J.) , 2020 | Pubmed ID: 32524487
Morphological variability is greater at developing than mature mouse neuromuscular junctions.
Journal of anatomy Jun, 2020 | Pubmed ID: 32533580
A video protocol for rapid dissection of mouse dorsal root ganglia from defined spinal levels.
BMC research notes Jun, 2020 | Pubmed ID: 32580748
Developmental demands contribute to early neuromuscular degeneration in CMT2D mice.
Cell death & disease 07, 2020 | Pubmed ID: 32703932
Knockin' on heaven's door: Molecular mechanisms of neuronal tau uptake.
Journal of neurochemistry 03, 2021 | Pubmed ID: 32770783
Altered Sensory Neuron Development in CMT2D Mice Is Site-Specific and Linked to Increased GlyRS Levels.
Frontiers in cellular neuroscience , 2020 | Pubmed ID: 32848623
Potential human transmission of amyloid β pathology: surveillance and risks.
The Lancet. Neurology 10, 2020 | Pubmed ID: 32949547
Walking the line: mechanisms underlying directional mRNA transport and localisation in neurons and beyond.
Cellular and molecular life sciences : CMLS Mar, 2021 | Pubmed ID: 33341920
Kidins220 deficiency causes ventriculomegaly via SNX27-retromer-dependent AQP4 degradation.
Molecular psychiatry May, 2021 | Pubmed ID: 34002021
NMJ-Analyser identifies subtle early changes in mouse models of neuromuscular disease.
Scientific reports 06, 2021 | Pubmed ID: 34112844
Dissection, in vivo imaging and analysis of the mouse epitrochleoanconeus muscle.
Journal of anatomy Jun, 2021 | Pubmed ID: 34121181
FUS-ALS mutants alter FMRP phase separation equilibrium and impair protein translation.
Science advances Jul, 2021 | Pubmed ID: 34290090
Exceptionally potent human monoclonal antibodies are effective for prophylaxis and treatment of tetanus in mice.
The Journal of clinical investigation 11, 2021 | Pubmed ID: 34618682
Nicolás Stuardo1,2,
Guillermo Moya-Alvarado1,3,
Carolina Ramírez1,3,
Giampietro Schiavo4,
Francisca C. Bronfman1,3
1Department of Physiology, Faculty of Biological Sciences, Pontificia Universidad Católica de Chile,
2Department of Molecular and Cellular Biology, Faculty of Biological Sciences, Center for Aging and Regeneration (CARE UC), Pontificia Universidad Católica de Chile,
3Institute of Biomedical Sciences. Faculty of Medicine and Faculty of Life Sciences, Universidad Andrés Bello,
4Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology and UK Dementia Research Institute at UCL, University College London Campus
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