Dr Sue Brown has been awarded a new grant, in collaboration with Professors Caroline Sewry (Institute of Neurology, London) and Glenn Morris (RJAH Orthopaedic Hospital, Oswestry) by the LGMD2I Research Fund, Samantha J Brazzo Foundation and Cure CMD.
α dystroglycan is a peripheral membrane glycoprotein present in association with the basement membrane of a diverse number of tissues. Defects in its glycosylation lead to a number of different forms of neuromuscular disease (termed dystroglycanopathies). However, the glycosylation pattern of α-dystroglycan is complex and limited amounts of biopsy material suggest that immunolabelling for different epitopes might be more informative than a single antibody when evaluating patients with a different clinical phenotype.
This grant has been awarded to test a panel of newly generated mononclonal antibodies on human muscle biopsies and animal models with the ultimate aim of using them in a clinical setting. This will require the conditions to be standardized with respect to fixation, permeabilisation and detection method in order that they might be used worldwide for both diagnostics and research. They also plan to evaluate α-dystroglycan by Western blot which would allow us to compare the relative molecular mass of the core protein in different patient groups – something which has been problematic in the past.