Prenatal muscle development in a mouse model for the secondary dystroglycanopathies

Kim, J; Hopkinson, M; Kavishwar, M; Fernandez-Fuente, M; Brown, S C

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.

Citation

Kim, J., Hopkinson, M., Kavishwar, M., Fernandez-Fuente, M., & Brown, S. C. (2016). Prenatal muscle development in a mouse model for the secondary dystroglycanopathies. Skeletal Muscle, 6(3), https://doi.org/10.1186/s13395-016-0073-y

• Journal Article Type: Article
• Acceptance Date: Jan 5, 2016
• Publication Date: Feb 19, 2016
• Deposit Date: Feb 20, 2016
• Publicly Available Date: Feb 20, 2016
• Journal: Skeletal Muscle
• Electronic ISSN: 2044-5040
• Publisher: BioMed Central
• Peer Reviewed: Peer Reviewed
• Volume: 6
• Issue: 3
• DOI: https://doi.org/10.1186/s13395-016-0073-y
• Public URL: https://rvc-repository.worktribe.com/output/1397599