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Dr James Sleigh
513
Queen Square House
Institute of Neurology, Queen Square
London
WC1N 3BG
Tel: +44(0)2034484334
Appointment
  • Senior Research Fellow
  • Department of Neuromuscular Diseases
  • UCL Queen Square Institute of Neurology
  • Faculty of Brain Sciences
Biography

I received my 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). I then completed my MRC-funded DPhil at the University of Oxford (2009-2012), extending my work on SMA, while studying a number of other disorders impacting peripheral motor and sensory nerves. From 2012-2014, I worked in the laboratory of Dr. Zameel Cader on the genetic peripheral neuropathy Charcot-Marie-Tooth disease type 2D (CMT2D). During a four year, Wellcome Trust-funded Sir Henry Wellcome Postdoctoral Fellowship (2014-2018), I continued my research on CMT2D disease mechanisms at the UCL Queen Square Institute of Neurology working with Prof. Giampietro Schiavo. I am now a Senior Research Fellow in the newly formed Department of Neuromuscular Diseases.

Research Themes
Research Summary

The fundamental question driving my research is how do mutations in widely expressed genes cause selective peripheral nerve deterioration? Mutations in many different genes important throughout the body manifest in a very specific detrimental effect on motor and sensory nerves, and I want to better understand the molecular and cellular mechanisms causing this. Charcot-Marie-Tooth disease (CMT) is a large group of genetically diverse peripheral neuropathies that share the principal pathological feature of progressive motor and sensory impairment. CMT type 2D (CMT2D) is caused by dominant, toxic gain-of-function mutations in a gene called GARS, which encodes glycyl-tRNA synthetase (GlyRS). GlyRS attaches the amino acid glycine to its partner transfer RNA (tRNA), thereby priming the tRNA for protein translation. This housekeeping function of glycine aminoacylation explains the widespread and constitutive nature of GARS expression, but how do mutations that affect a protein found in all cells selectively trigger peripheral nerve degeneration?


Using CMT2D as a paradigm, I am studying causes of motor and sensory nerve degeneration and the mechanisms underpinning the dynamic process of axonal transport. By improving understanding of disease pathways and pathologies, I aim to generate effective, pre-clinical gene therapies for genetic peripheral nerve diseases

Appointments
01-OCT-2014 – 30-SEP-2018 Sir Henry Wellcome Postdoctoral Fellow Sobell Department of Motor Neuroscience University College London Institute of Neurology, United Kingdom
01-NOV-2012 – 30-SEP-2014 Postdoctoral Research Scientist Nuffield Department of Clinical Neurosciences University of Oxford, United Kingdom
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