Nature Communications (May 2021)
FKRP-dependent glycosylation of fibronectin regulates muscle pathology in muscular dystrophy
- A. J. Wood,
- C. H. Lin,
- M. Li,
- K. Nishtala,
- S. Alaei,
- F. Rossello,
- C. Sonntag,
- L. Hersey,
- L. B. Miles,
- C. Krisp,
- S. Dudczig,
- A. J. Fulcher,
- S. Gibertini,
- P. J. Conroy,
- A. Siegel,
- M. Mora,
- P. Jusuf,
- N. H. Packer,
- P. D. Currie
Affiliations
- A. J. Wood
- Australian Regenerative Medicine Institute (ARMI), Faculty of Medicine, Nursing and Health Science, Monash University
- C. H. Lin
- Biomolecular Discovery and Design Research Centre, Department of Molecular Sciences, Faculty of Science & Engineering, Macquarie University
- M. Li
- Australian Regenerative Medicine Institute (ARMI), Faculty of Medicine, Nursing and Health Science, Monash University
- K. Nishtala
- Biomolecular Discovery and Design Research Centre, Department of Molecular Sciences, Faculty of Science & Engineering, Macquarie University
- S. Alaei
- Australian Regenerative Medicine Institute (ARMI), Faculty of Medicine, Nursing and Health Science, Monash University
- F. Rossello
- Australian Regenerative Medicine Institute (ARMI), Faculty of Medicine, Nursing and Health Science, Monash University
- C. Sonntag
- Australian Regenerative Medicine Institute (ARMI), Faculty of Medicine, Nursing and Health Science, Monash University
- L. Hersey
- Australian Regenerative Medicine Institute (ARMI), Faculty of Medicine, Nursing and Health Science, Monash University
- L. B. Miles
- Australian Regenerative Medicine Institute (ARMI), Faculty of Medicine, Nursing and Health Science, Monash University
- C. Krisp
- Biomolecular Discovery and Design Research Centre, Department of Molecular Sciences, Faculty of Science & Engineering, Macquarie University
- S. Dudczig
- Australian Regenerative Medicine Institute (ARMI), Faculty of Medicine, Nursing and Health Science, Monash University
- A. J. Fulcher
- Monash Micro Imaging Facility, Monash University
- S. Gibertini
- Muscle Cell Biology Laboratory, Neuromuscular Diseases and Neuroimmunology Unit, Fondazione IRCCS Istituto Neurologico “Carlo. Besta” Via Temolo 4
- P. J. Conroy
- Department of Biochemistry and Molecular Biology, Faculty of Medicine, Nursing and Health Science, Monash University
- A. Siegel
- Australian Regenerative Medicine Institute (ARMI), Faculty of Medicine, Nursing and Health Science, Monash University
- M. Mora
- Muscle Cell Biology Laboratory, Neuromuscular Diseases and Neuroimmunology Unit, Fondazione IRCCS Istituto Neurologico “Carlo. Besta” Via Temolo 4
- P. Jusuf
- Australian Regenerative Medicine Institute (ARMI), Faculty of Medicine, Nursing and Health Science, Monash University
- N. H. Packer
- Biomolecular Discovery and Design Research Centre, Department of Molecular Sciences, Faculty of Science & Engineering, Macquarie University
- P. D. Currie
- Australian Regenerative Medicine Institute (ARMI), Faculty of Medicine, Nursing and Health Science, Monash University
- DOI
- https://doi.org/10.1038/s41467-021-23217-6
- Journal volume & issue
-
Vol. 12,
no. 1
pp. 1 – 12
Abstract
FKRP mutations cause muscular dystrophies with varied clinical presentations. The target of FKRP is α-dystroglycan, but here the authors show that FKRP also directs sialylation of fibronectin, a process that is essential for recruitment o collagen to the muscle basement membrane.
