Job: Reader in Cell Biology
Faculty: Health and Life Sciences
School/department: Leicester School of Pharmacy
Address: De Montfort University, The Gateway, Leicester, LE1 9BH.
T: +44 (0)116 2576368
The Glycosphingolipid (GSL) storage diseases are a group of diseases where GSLs accumulate in tissues due to a defect in their lysosomal breakdown. GSL storage diseases have varying degrees of central nervous system involvement and in Tay-Sachs and Sandhoff diseases the neuropathology is very severe and frequently lethal in early infancy.
How lysosomal storage of GSL causes such severe neuropathology is not known at present. A strategy that can be applied to treat these diseases is substrate deprivation.
This approach aims to balance the rate of GSL biosynthesis with the impaired rate of catabolism, thus preventing accumulation of GSLs. Two imino sugar inhibitors of Glucosylceramide synthase, which catalyses the first step in GSL synthesis.
How does the storage of lipids lead to disease?
What are the normal functions of the lipids that accumulate?
Click here for a full listing of Dan Sillence‘s publications and outputS.
BSc PhD (Biochemistry, Dundee)
Peter Jones Carlton memorial award (2006) for new therapy for Niemann-Pick C disease with Emyr Lloyd-Evans and Prof Frances Platt, University of Oxford.
Sphingolipid cell biology, Unilever UK.
Assay of sphingomyelinase inhibitors, Off-Label Ltd/Penn Ltd, research project, July 2008 – Nov 2008, PI.
Sphingolipid cell biology, Startup – 1/1/2008 – 2009, PI.
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