Muscular dystrophies, the cytoskeleton and cell adhesion.
Muscular dystrophies are associated with mutations in genes encoding several classes of proteins. These range from extracellular matrix and integral membrane proteins to cytoskeletal proteins, but also include a heterogeneous group of proteins including proteases, nuclear proteins, and signalling molecules. Muscular dystrophy phenotypes have also become evident in studies on various knockout mice defective in proteins not previously considered or known to be mutated in muscular dystrophies. Some unifying themes are beginning to emerge from all of these data. This review will consider recent advances in our understanding of the molecules involved and bring together data that suggest a role for the cytoskeleton and cell adhesion in muscular dystrophies.
Spence
Heather J
HJ
Institute of Biological and Life Sciences, Glasgow Cell Biology Group, Scotland.
Chen
Yun-Ju
YJ
Winder
Steven J
SJ
D000818:Animals; D002448:Cell Adhesion; D003599:Cytoskeleton / Q000378:metabolism*; D016189:Dystrophin / Q000378:metabolism; D006023:Glycoproteins / Q000378:metabolism; D016023:Integrins / Q000378:metabolism; D007382:Intermediate Filaments / Q000378:metabolism; D051379:Mice; D018345:Mice, Knockout; D008954:Models, Biological; D018482:Muscle, Skeletal / Q000378:metabolism; D009136:Muscular Dystrophies / Q000235:genetics* / Q000473:pathology*; D009154:Mutation; D010641:Phenotype
D016189:Dystrophin; D006023:Glycoproteins; D016023:Integrins