The physical state of the erythrocyte membrane in myotonic dystrophy. 1989

F Beaugé, and J Gallay, and O Sydow, and H Stibler
Unité de Neurotoxicologie, INSERM U.26, Hôpital F. Widal, Paris, France.

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.

UI MeSH Term Description Entries
D008297 Male Males
D008560 Membrane Fluidity The motion of phospholipid molecules within the lipid bilayer, dependent on the classes of phospholipids present, their fatty acid composition and degree of unsaturation of the acyl chains, the cholesterol concentration, and temperature. Bilayer Fluidity,Bilayer Fluidities,Fluidities, Bilayer,Fluidities, Membrane,Fluidity, Bilayer,Fluidity, Membrane,Membrane Fluidities
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009136 Muscular Dystrophies A heterogeneous group of inherited MYOPATHIES, characterized by wasting and weakness of the SKELETAL MUSCLE. They are categorized by the sites of MUSCLE WEAKNESS; AGE OF ONSET; and INHERITANCE PATTERNS. Muscular Dystrophy,Myodystrophica,Myodystrophy,Dystrophies, Muscular,Dystrophy, Muscular,Myodystrophicas,Myodystrophies
D004912 Erythrocytes Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN. Blood Cells, Red,Blood Corpuscles, Red,Red Blood Cells,Red Blood Corpuscles,Blood Cell, Red,Blood Corpuscle, Red,Erythrocyte,Red Blood Cell,Red Blood Corpuscle
D005260 Female Females
D005456 Fluorescent Dyes Chemicals that emit light after excitation by light. The wave length of the emitted light is usually longer than that of the incident light. Fluorochromes are substances that cause fluorescence in other substances, i.e., dyes used to mark or label other compounds with fluorescent tags. Flourescent Agent,Fluorescent Dye,Fluorescent Probe,Fluorescent Probes,Fluorochrome,Fluorochromes,Fluorogenic Substrates,Fluorescence Agents,Fluorescent Agents,Fluorogenic Substrate,Agents, Fluorescence,Agents, Fluorescent,Dyes, Fluorescent,Probes, Fluorescent,Substrates, Fluorogenic
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

Related Publications

F Beaugé, and J Gallay, and O Sydow, and H Stibler
April 1987, Clinica chimica acta; international journal of clinical chemistry,
F Beaugé, and J Gallay, and O Sydow, and H Stibler
June 1980, Journal of the neurological sciences,
F Beaugé, and J Gallay, and O Sydow, and H Stibler
June 1985, Journal of neurochemistry,
F Beaugé, and J Gallay, and O Sydow, and H Stibler
August 1981, Journal of the neurological sciences,
F Beaugé, and J Gallay, and O Sydow, and H Stibler
June 1980, The Journal of clinical investigation,
F Beaugé, and J Gallay, and O Sydow, and H Stibler
May 1982, Journal of the neurological sciences,
F Beaugé, and J Gallay, and O Sydow, and H Stibler
October 1981, Journal of the neurological sciences,
F Beaugé, and J Gallay, and O Sydow, and H Stibler
January 1982, Journal of the neurological sciences,
F Beaugé, and J Gallay, and O Sydow, and H Stibler
February 1985, Experimental neurology,
F Beaugé, and J Gallay, and O Sydow, and H Stibler
January 1975, The Journal of membrane biology,
Copied contents to your clipboard!