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.
| Item Type: | Article |
|---|---|
| RVC Publication Type: | Research (full) paper |
| WoS ID: | 000370410900001 |
| DOI: | https://doi.org/10.1186/s13395-016-0073-y |
| Departments: | Comparative Biomedical Sciences |
| Research Programmes: | Comparative Physiology & Medicine > Musculoskeletal Biology |
| Depositing User: | RVC Auto-import |
| Last Modified: | 21 Nov 2020 04:43 |
| URI: | https://researchonline.rvc.ac.uk/id/eprint/10048 |
| Date Deposited: | 20 February 2016 |
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