Prenatal muscle development in a mouse model for the secondary dystroglycanopathies

Kim, J and Hopkinson, M and Kavishwar, M and Fernandez-Fuente, M and Brown, S C (2016) Prenatal muscle development in a mouse model for the secondary dystroglycanopathies. Skeletal Muscle, 6 (3).

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Abstract

The defective glycosylation of α-dystroglycan is associated with a group of muscular dystrophies that are collectively referred to as the secondary dystroglycanopathies. Mutations in the gene encoding fukutin-related protein (FKRP) are one of the most common causes of secondary dystroglycanopathy in the UK and are associated with a wide spectrum of disease. Whilst central nervous system involvement has a prenatal onset, no studies have addressed prenatal muscle development in any of the mouse models for this group of diseases. In view of the pivotal role of α-dystroglycan in early basement membrane formation, we sought to determine if the muscle formation was altered in a mouse model of FKRP-related dystrophy.

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