Paucity of deleted mitochondrial DNAs in brain regions of Huntington's disease patients

Xi Chen, Eduardo Bonilla, Monica Sciacco, Eric A. Schon

Research output: Contribution to journalArticlepeer-review


Mitochondrial DNA deletions (Δ-mtDNAs), originally found at high levels in patients with sporadic mitochondrial encephalomyopathies, have also been found to accumulate at extremely low levels during normal human aging, especially in long-lived postmitotic tissues such as muscle and brain. We have now quantitated the amount of one such Δ-mtDNA species, the so-called 'common deletion', in brain regions from patients with Huntington's disease (HD). Surprisingly, we found a marked decrease in the amount of this Δ-mtDNA in the occipital cortex and putamen as compared to age-matched controls; however, no change was found in caudate. Using immunohistochemistry of brain sections, we found no differences in the staining pattern for selected respiratory chain polypeptides between the HD and control tissues. The reduction in the amount of Δ-mtDNAs in HD may be related in part to the astrocytic gliosis in the affected areas, in which the deletion-rich neurons are replaced by relatively deletion-poor astrocytes.

Original languageEnglish
Pages (from-to)229-233
Number of pages5
JournalBiochimica et Biophysica Acta - Molecular Basis of Disease
Issue number1
Publication statusPublished - May 24 1995


  • Aging
  • Common deletion
  • Electron transport chain
  • GFAP
  • Glial fibrillary acidic protein
  • Mitochondrion
  • mtDNA

ASJC Scopus subject areas

  • Molecular Biology
  • Molecular Medicine
  • Biophysics


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