UCL  IRIS
Institutional Research Information Service
UCL Logo
Please report any queries concerning the funding data grouped in the sections named "Externally Awarded" or "Internally Disbursed" (shown on the profile page) to your Research Finance Administrator. Your can find your Research Finance Administrator at https://www.ucl.ac.uk/finance/research/rs-contacts.php by entering your department
Please report any queries concerning the student data shown on the profile page to:

Email: portico-services@ucl.ac.uk

Help Desk: http://www.ucl.ac.uk/ras/portico/helpdesk
 More search options
Dr James Sleigh
513
Queen Square House
Institute of Neurology, Queen Square
London
WC1N 3BG
Tel: +44(0)2034484334
Appointment
  • Sir Henry Wellcome Postdoctoral Fellow
  • Motor Neurosci & Mov Disorders
  • 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). I am now a Wellcome Trust-funded Sir Henry Wellcome Postdoctoral Fellow continuing my work on the mechanisms underlying CMT2D.

Research Themes
Research Summary

The fundamental question at the centre of my research is how do mutations in widely expressed genes cause highly selective peripheral nerve deterioration? Mutations in numerous 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 cognate 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?


I currently have a Wellcome Trust Sir Henry Wellcome Postdoctoral Fellowship that I am using to answer this question. I am using live imaging of cellular dynamics, including axonal transport, to interrogate both the motor and sensory systems in cell and animal models to define the impaired pathways linking GARS mutations to peripheral neurodegeneration.

Appointments
01-NOV-2012 – 30-SEP-2014 Postdoctoral Research Scientist Nuffield Department of Clinical Neurosciences University of Oxford, United Kingdom
Please report any queries concerning the data shown on this page to https://www.ucl.ac.uk/hr/helpdesk/helpdesk_web_form.php
University College London - Gower Street - London - WC1E 6BT Tel:+44 (0)20 7679 2000

© UCL 1999–2011

Search by