Dystrophin as a mechanochemical transducer in skeletal muscle.
This review is primarily concerned with two key issues in research on dystrophin: (1) how the protein interacts with the plasma membrane of skeletal muscle fibres and (2) how an absence of dystrophin gives rise to Duchenne muscular dystrophy. In relation to the first point, we suggest that the post-translational acylation of dystrophin may contribute to its interaction with the plasma membrane. Regarding the second point, it is generally considered that an absence of dystrophin makes the plasma membrane susceptible to damage by contraction/relaxation cycles. In this connection, we propose that the progressive nature of Duchenne dystrophy, and the phenotypic characteristics of mdx mice, are more consistent with dystrophin functioning as a mechanical transducer that transmits growth stimuli from the enlarging skeleton to the muscle. On the basis of this hypothesis, dystrophin-deficient muscles would be unable to grow at the same rate as the skeleton.
Brown
S C
SC
Department of Biochemistry and Chemistry, Royal Free Hospital School of Medicine, London, UK.
Lucy
J A
JA
D000595:Amino Acid Sequence; D000818:Animals; D002462:Cell Membrane / Q000502:physiology / Q000648:ultrastructure; D016189:Dystrophin / Q000737:chemistry / Q000235:genetics / Q000502:physiology*; D006801:Humans; D008961:Models, Structural; D008969:Molecular Sequence Data; D024510:Muscle Development; D009132:Muscles / Q000502:physiology*; D017386:Sequence Homology, Amino Acid; D015398:Signal Transduction
D016189:Dystrophin