Electron spin resonance investigations of membrane proteins in erythrocytes in muscle diseases. Duchenne and myotonic muscular dystrophy and congenital myotonia.
Comparison of electron spin resonance spectra of spin labeled erythrocyte membranes from patients with the dystrophic conditions Duchenne and myotonic muscular dystrophy with those of normal controls suggests that alterations in membrane protein conformation and/or organization are present in these disease states. These protein alterations are not apparent in the non-dystrophic disease congenital myotonia. The results suggest a correlation between changes in the physical state of proteins in membranes with the presence of dystrophy. In addition, the present results from erythrocytes lend support for the concept of a generalized membrane defect in these diseases.
D004578:Electron Spin Resonance Spectroscopy; D004910:Erythrocyte Membrane / Q000502:physiology* / Q000648:ultrastructure; D004912:Erythrocytes / Q000502:physiology*; D006801:Humans; D008565:Membrane Proteins / Q000097:blood; D009136:Muscular Dystrophies / Q000097:blood*; D009224:Myotonia Congenita / Q000097:blood*; D009223:Myotonic Dystrophy / Q000097:blood*; D011487:Protein Conformation; D013113:Spin Labels
D008565:Membrane Proteins; D013113:Spin Labels