Muscular Dystrophy UK has named Senior Research Fellow Dr James Sleigh as the winner of its inaugural Research Image Competition.
Dr James Sleigh, who is based at the Institute of Neurology, University College London, dedicates his research to understanding how motor and sensory neurons are preferentially affected by genetic mutations that cause the condition Charcot-Marie-Tooth disease (CMT). He hopes this work will eventually lead to developing a gene therapy that can help treat the condition.
Charcot-Marie-Tooth disease is a progressive, inherited condition that causes problems in nerves controlling movement of the hands and legs, and in some cases, chronic pain and tiredness. This is because motor and sensory neurons become damaged.
Dr James Sleigh’s winning image, which was selected by Muscular Dystrophy UK supporters, is part of a study into CMT Type 2D, and shows a collection of sensory nerves called dorsal root ganglion (DRG), which are found in pairs along the spinal cord.
Dr James Sleigh, from London, explained:
DRG receive information from the external environment via long, thin, cable-like extensions called axons. DRG were extracted and very thin, 10 micrometre sections were cut through the tissue. Sections were then collected onto microscope slides and stained using a technique known as immuno-histochemistry.
“This process relies on antibodies that very specifically recognise particular proteins. In the case of the image, fluorescent antibodies against two different proteins found in sensory nerves were used, as depicted by the green, red and yellow, as well as a fluorescent dye that recognises DNA, which is highlighted by the blue. Using a microscope called a confocal microscope, I took pictures of the DRG to identify exactly where the proteins and DNA could be found within the sensory nerves.”
Through his work, Dr James Sleigh found that the sensory neurons in CMT Type 2D did not develop properly. This has led to a greater understanding of the disease process in CMT Type 2D and why only neurons are affected. The work also highlighted a possible therapeutic strategy for the condition.
CMT Type 2D is caused by mutations in the GARS gene, which results in the production of a faulty GARS protein,” said Dr James Sleigh. “Although this protein is found in all cells of the body, only the nervous system is affected by CMT Type 2D. The exact reason for this remains a mystery, but my research group is making strides into understanding the impaired genetic pathways that lead to the condition.”
He added: “There is currently no cure or treatment for CMT. Our work in trying to understand the mechanisms underlying CMT Type 2D has the very real potential of generating gene therapies that may be able to treat or at least halt progression of the condition.”