Huntington's disease: A generalized membrane defect

D. Allan Butterfield, William R. Markesbery

Research output: Contribution to journalArticlepeer-review

24 Scopus citations


Huntington's disease, a progressively degenerative neurological disorder inherited as an autosomal dominant trait, results in selective neuronal loss in the basal ganglia and other areas of the brain. Based on research in our laboratory employing electron spin resonance, analytical, enzymatic, biochemical and morphological techniques to study erythrocyte membranes, which are completely outside the central nervous system, we have suggested that Huntington's disease is associated with a generalized membrane defect involving a protein and probably manifested at the external membrane surface. Other workers have subsequently obtained biophysical, biochemical, and morphological results on extraneural tissue in Huntington's disease including erythrocytes, lymphocytes, platelets,and cultured skin fibroblasts that supports this hypothesis. This review will summarize and evaluate the current knowledge of the involvement of a membrane defect in the etiology and pathogenesis of Huntington's disease.

Original languageEnglish
Pages (from-to)1117-1131
Number of pages15
JournalLife Sciences
Issue number10
StatePublished - Mar 9 1981

Bibliographical note

Funding Information:
This work was supported in part by grants from NIH (NS-14221) and the Muscular Dystrophy Association of America.

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology (all)
  • Pharmacology, Toxicology and Pharmaceutics (all)


Dive into the research topics of 'Huntington's disease: A generalized membrane defect'. Together they form a unique fingerprint.

Cite this