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Citation

Thesis Title
Structural and Biochemical Characterisation of Utrophin and Dystrophin Spectrin Repeat Domains

Read article at Massey Research Online:

Duchenne and Becker muscular dystrophies are muscle-wasting disorders caused by mutations in the X-linked dystrophin gene. Dystrophin is a large cytoskeletal protein belonging to the spectrin superfamily, that links intracellular F-actin to the extracellular matrix聽via聽a membrane-associated glyco-protein complex thus maintaining structural rigidity and flexibility. Utrophin is a widely expressed protein that has been shown to functionally compensate for dystrophin in cultured muscle cells as well as in the muscular dystrophy mice model. Therapeutic strategies to replace individuals having defective dystrophin with utrophin require full characterisation of these proteins.

Supervisors
Associate Professor Andrew Sutherland-Smith
Associate Professor Steven Pascal