FKRP-dependent glycosylation of fibronectin regulates muscle pathology in muscular dystrophy
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.
Saved in:
Main Authors: | 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 |
---|---|
Format: | article |
Language: | EN |
Published: |
Nature Portfolio
2021
|
Subjects: | |
Online Access: | https://doaj.org/article/5e6f8208fa384dfab7d8162c046eec48 |
Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
Similar Items
-
Crystal structures of fukutin-related protein (FKRP), a ribitol-phosphate transferase related to muscular dystrophy
by: Naoyuki Kuwabara, et al.
Published: (2020) -
Ribitol restores functionally glycosylated α-dystroglycan and improves muscle function in dystrophic FKRP-mutant mice
by: Marcela P. Cataldi, et al.
Published: (2018) -
Hsp90 binds directly to fibronectin (FN) and inhibition reduces the extracellular fibronectin matrix in breast cancer cells.
by: Morgan C Hunter, et al.
Published: (2014) -
ISPD produces CDP-ribitol used by FKTN and FKRP to transfer ribitol phosphate onto α-dystroglycan
by: Isabelle Gerin, et al.
Published: (2016) -
Role of fibronectin in the adhesion of Acinetobacter baumannii to host cells.
by: Younes Smani, et al.
Published: (2012)