Changes in mitochondrial complex I activity and coenzyme Q binding site in Leber's hereditary optic neuropathy (LHON)

A. Ghelli, M. Degli Esposti, V. Carelli, G. Lenaz

Research output: Contribution to journalArticlepeer-review

Abstract

The complex I function in sub-mitochondrial particles was studied in platelets from patients and healthy carriers with 11778/ND4 or 3460/ND1 mtDNA point mutations associated with LHON. Both 11778/ND4 and 3460/ND1 mutations induced rotenone resistance and 11778/ND4 showed an increased K(m) for ubiquinol-2 with respect to the control group. It was concluded that even with different pathogenic mechanisms both mutations affect the quinone binding site of complex I.

Original languageEnglish
JournalMolecular Aspects of Medicine
Volume18
Issue numberSUPPL.
DOIs
Publication statusPublished - 1997

Keywords

  • Complex I
  • LHON
  • mtDNA mutations
  • Platelets
  • Rotenone

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Molecular Medicine

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