title: The physical state of the erythrocyte membrane in myotonic dystrophy.
abstract: The molecular pathology of myotonic dystrophy is believed to be expressed at the plasma membrane level. Previous assessments of membrane fluidity, a marker of the biochemical state of the membrane, have yielded conflicting results. In this study, erythrocyte membrane fluidity was reevaluated using highly sensitive fluorescence probe techniques. Steady-state anisotropy was measured with diphenylhexatriene (DPH), trimethylaminophenyl-hexatriene (TMA-DPH) and phenylhexatrienylphenylpropionic acid, probing different regions of the membrane. In the patients, significantly increased steady-state anisotropy was obtained with DPH, probing the hydrophobic core of the membrane, while slightly reduced anisotropy was found with TMA-DPH. The dynamic properties of the membrane lipids were further examined by means of time-resolved measurements with DPH. The excited state decay kinetics could best be described by a bi-exponential decay model. A large redistribution of the probe populations and a reduction of the average order parameter were found in the patients indicating a less ordered or more fluid lipid matrix. These perturbations might be induced by a protein abnormality and altered protein-lipid interaction within the erythrocyte membrane.
authors: Unité de Neurotoxicologie, INSERM U.26, Hôpital F. Widal, Paris, France.
authors: F
authors: F
authors: Beaugé
authors: J
authors: J
authors: Gallay
authors: O
authors: O
authors: Sydow
authors: H
authors: H
authors: Stibler
mesh_terms: D000293:Adolescent; D000328:Adult; D004912:Erythrocytes / Q000473:pathology*; D005260:Female; D005456:Fluorescent Dyes; D006801:Humans; D008297:Male; D008560:Membrane Fluidity; D008875:Middle Aged; D009136:Muscular Dystrophies / Q000097:blood*
chemical_list: D005456:Fluorescent Dyes