Electron spin resonance, hematological, and deformability studies of erythrocytes from patients with Huntington's disease

D. Allan Butterfield, Mark J. Purdy, William R. Markesbery

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24 Scopus citations

Abstract

Electron spin resonance, hematologic, and deformability studies of erythrocytes from patients with Huntington's disease have been performed. A decreased deformability of Huntington's disease erythrocytes compared to normal controls was demonstrated. No difference in erythrocyte hematologic indices, osmotic fragility, reticulocyte counts, or intracellular Na+ concentration was found. Huntington's disease serum had no demonstrable effect on electron spin resonance parameters of a protein-specific spin label attached to membrane proteins in control erythrocytes compared to the effect of control serum. This finding suggests that under the conditions employed no serum component or circulating factor is responsible for the changes in the physical state of membrane proteins in Huntington's disease erythrocytes (Butterfield, D.A., Oeswein, J.Q. and Markesbery, W.R. (1977) Nature 267, 453-455). No alteration in lipid fluidity of Huntington's disease erythrocyte membranes could be discerned suggesting that the underlying molecular defect in Huntington's disease involves a membrane protein. The results of the present studies on erythrocytes strongly support the concept that Huntington's disease is associated with a generalized membrane abnormality.

Original languageEnglish
Pages (from-to)452-458
Number of pages7
JournalBBA - Biomembranes
Volume551
Issue number2
DOIs
StatePublished - Mar 8 1979

Bibliographical note

Funding Information:
concept that Huntington's disease is associated with a diffuse membrane defect. We thank Dr. Jon Gockerman, Department of Medicine of the University of Kentucky Medical Center, for help and advice with the hematologic aspects of this study and Marcia Butterfield for technical assistance. This work was supported in part by the National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health (NS 13791-01A1) (D.A.B.) and NS-14221-01 (W.R.M.), the Muscular Dystrophy Association of America (D.A.B.), and the Graduate School of the University of Kentucky (D.A.B.).

Keywords

  • (Erythrocyte membranes)
  • Deformability
  • ESR
  • Fluidity
  • Huntington's disease
  • Membrane defect

ASJC Scopus subject areas

  • Biophysics
  • Biochemistry
  • Cell Biology

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