A spin label study of the erythrocyte membranes in duchenne muscular dystrophy

Laurent, Michel; Daveloose, Denis; Leterrier, F; Fischer, Siegmund; Schapira, G

doi:10.1016/0009-8981(80)90460-x

Cited by 30 publications

(10 citation statements)

References 16 publications

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“…The 16 DSA spin probe explores the hydrophobic core of the membrane and has been successfully utilized to study RBC thermotropic properties [ 14,15,22,25,. This label is preferentially incorporated into the membrane and we observed that the label detectable in the supernatant of the cell hemolysate was shaped as expected for a free label and was only a small percentage (< 1 %) of the membrane-bound label.…”

Section: Analysis Of Epr Spectra Of Intact Rbcmentioning

confidence: 76%

“…The discontinuities can be described as break points of the regression lines presented. Thermotropic breaks are present at 8, 20, and 40" f 1.5"C. EPR studies reported previously [14,22,25,27,37] showed the presence in the 0-30°C range of only one thermotropic break apparently overlooking one of the low temperature breaks. A more accurate analysis revealed the presence of two thermotropic breaks in the 0-30°C range.…”

Section: Analysis Of Epr Spectra Of Intact Rbcmentioning

confidence: 93%

“…Spin-labeled stearic acids have been shown to be convenient to study RBC structural transitions [ 14,15,22,25,. The main question that arises from these studies is the meaning of the thermotropic transitions detected by this technique.…”

Section: Jcbmentioning

confidence: 99%

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Characterization of thermotropic structural transitions of the erythrocyte membrane: A biochemical and electron‐paramagnetic resonance approach

Minetti

Ceccarini

Maria

et al. 1984

J of Cellular Biochemistry

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The relationship between membrane structural properties and functions has been generally inferred from observed thermotropic phenomena. By the use of 16-dinyloxyl stearic acid spin probe we investigated the red blood cell membrane components involved in three characteristic thermotropic structural transitions occurring at 8, 20, and 40 degrees C. The transition at 8 degrees C is removed by chymotrypsin treatment at the cytoplasmic membrane layer. The 20 degrees C phase transition is unmodified after chymotrypsin treatment and occurs at 15 degrees C after complete proteolysis of intramembrane chymotrypsin-insensitive peptides. Liposomes from the total lipid extract of RBC show only one thermotropic transition at 15 degrees C. The 40 degrees C phase transition is absent in vesicles free of skeletal proteins, in vesicles obtained after RBC storage, and in low-ionic-strength resealed ghosts. Transitions at 8 degrees C and 40 degrees C appear to be due to the interactions of cytoplasmic exposed proteins with membrane, whereas the 20 degrees C transition is intrinsic to the lipid component.

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Section: Analysis Of Epr Spectra Of Intact Rbcmentioning

confidence: 76%

Section: Analysis Of Epr Spectra Of Intact Rbcmentioning

confidence: 93%

See 1 more Smart Citation

Characterization of thermotropic structural transitions of the erythrocyte membrane: A biochemical and electron‐paramagnetic resonance approach

Minetti

Ceccarini

Maria

et al. 1984

J of Cellular Biochemistry

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“…Boule et al (5) Since the reduced cell number and DNA amount on day 7 and the increased doubling time in DMD fibroblasts could be the result of an alteration in the plasma membrane, and since many reports suggest a membrane defect in DMD for various cells (2,6,13,18,20,23,29,30,39,41), we studied the activity of the membrane bound enzyme 5'-nucleotidase. We found a significant increase of its specific activity in the DMD cells.…”

Section: Discussionmentioning

confidence: 99%

Abnormal Growth Kinetics and 5′-Nucleotidase Activities in Cultured Skin Fibroblasts from Patients with Duchenne Muscular Dystrophy

et al. 1981

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SummaryThe experiments reported herein compare growth kinetics and biochemical properties of cultured skin fibroblasts from patients with Duchenne muscular dystrophy (DMD) and matched normal controls.On day 7 after plating (6000 cells/cm2) cell number and DNA per dish are significantly reduced ( P < 0.001) in the cultures from DMD patients (n = 14)' compared to those from controls (n = 10). Moreover DMD cells contain less lipids and proteins per dish but more per cell than normal fibroblasts (not significant). Variations of media (McCoy's medium instead of Eagle's minimum essential medium) and sera (human cord serum instead of fetal calf serum) resulted in the same differences between DMD and control cells.Cell kinetic experiments (plating density: 2000 cells/cm2) show increased doubling times of DMD fibroblasts ( P < 0.001; ~D M D = 5; n,o,t,o~s = 4) whereas plating efficiency is equal for both DMD and controls.On day 7 the activity of the membrane bound enzyme 9-nucleotidase either per mg protein or per pg DNA is significantly elevated in cells from DMD patients ( P < 0.0005; nDMD = 8; nCont,,ls = 9) independent of cell density. Thus all findings in cultured DMD fibroblasts: increased doubling time, tendency to more voluminous cells, and elevated 5'-nucleotidase activity per cell suggest, that the DMD cells behave similar to prematurely aging cells. Until now we were not able to check whether any alterations of the plasma membrane are inducing early senescence or, reversely, premature aging is the cause of the postulated membrane alterations.If these findings were to be confirmed in cultured amniotic cells 13, 27, 40). All these findings are supposed to be secondary or tertiary alterations. The underlying metabolic defect is not yet known. Cultured fibroblasts obtained from explants of human skin biopsies have proved to be very useful for the investigations of numerous inborn errors of metabolism. Studies of cultured fibroblasts from DMD patients have been initiated only very recently. There are reports of altered morphology (43), reduced cell aggregation (17), decreased protein synthesis (5, 29), and decreased lysosomal dipeptidyl aminopeptidase I activity (1 1).Herein we report the results of investigations on cultured fibroblasts from patients with DMD: plating efficiency, doubling time, effects of different media and sera, 14C-thymidine incorporation in DNA, and activity of the membrane bound enzyme 5'-nucleotidase (EC 3.1.3.5.). MATERIALS AND METHODS MATERIALWe studied fibroblasts growing from skin biopsies of 14 patients with DMD, aged 4 to 14 yr, and of 10 normal boys matched by age, who were undergoing surgical treatment. The cells were usually cultured in Eagle's minimum essential medium (MEM), supplemented with nonessential amino acids and 10% fetal calf serum (FCS). To test the influence of different growth conditions, McCoy's 5A modified medium (26) was used instead of MEM, and MEM was supplemented with 10% human cord serum instead of FCS. METHODS Dfrom DMD fetuses, they could serve as a potential ...

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“…The lipid fluidity in the internal hydrophobic core of the lipid bilayer was measured in the presence of 16-DSA using an empirical parameter, the ratio of signal height, ho/h.l. This parameter reflects the fluidity of the lipid environment of the label (15). The spectrum of maleimide displays two classes of spin label binding sites: strongly immobilized (S sites) and weakly immobilized (W sites).…”

Section: Methodsmentioning

confidence: 99%

The effects of imidazoacridinones, new antineoplastic agents, on the human erythrocyte membrane

1997

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A spin label study of the erythrocyte membranes in duchenne muscular dystrophy

Cited by 30 publications

References 16 publications

Characterization of thermotropic structural transitions of the erythrocyte membrane: A biochemical and electron‐paramagnetic resonance approach

Characterization of thermotropic structural transitions of the erythrocyte membrane: A biochemical and electron‐paramagnetic resonance approach

Abnormal Growth Kinetics and 5′-Nucleotidase Activities in Cultured Skin Fibroblasts from Patients with Duchenne Muscular Dystrophy

The effects of imidazoacridinones, new antineoplastic agents, on the human erythrocyte membrane

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