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Prenatal muscle development in a mouse model for the secondary dystroglycanopathies

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

Authors

J Kim

M Hopkinson

M Kavishwar

M Fernandez-Fuente

S C Brown



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 Mar 29, 2024
Journal Skeletal Muscle
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

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