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The role of mitochondria in health, ageing, and diseases affecting vision
  1. A A Sadun1,
  2. V Carelli1,2
  1. 1Doheny Eye Institute, Departments of Ophthalmology and Neurological Surgery, Keck Medical School at USC, Los Angeles, CA, USA
  2. 2Department of Neurological Sciences, University of Bologna, Italy
  1. Correspondence to: Professor Alfredo Sadun Doheny Eye Institute, Departments of Ophthalmology and Neurological Surgery, Keck Medical School at USC, Los Angeles, CA, USA; asadun{at}usc.edu

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Control of mitochondrial mediated apoptosis will become a holy grail

Abu-Amero and Bosley have, in this issue (p 823),1 published a paper relating total cellular mitochondrial DNA (mtDNA) content with a classic neuro-ophthalmological disorder, non-arteritic anterior ischaemic optic neuropathy (NAION).2 NAION is widely regarded as a watershed ischaemic event to the optic nerve that occurs as a consequence of structural crowding in the optic disc (usually congenital) with compromise of the vascular supply (often from diabetes or hypertension).2,3

Mitochondria are intracellular organelles that carry their own peculiar genome, a circular 16.5 kb molecule that reflects their bacterial origin.4 Each cell carries a variable number of mitochondria and each mitochondrion has multiple mtDNA copies. Hence, cellular mtDNA copy number is a combination of these two variables; it is normalised for the nuclear DNA content that defines the number of cells sampled. Pathogenic mtDNA mutations have been associated with maternally inherited optic neuropathy—that is, Leber’s hereditary optic neuropathy (LHON).5 mtDNA copy number has been reported to be increased in LHON, possibly as a compensatory mechanism for the impaired mitochondrial respiration.6

Remarkably, Abu-Amero and Bosley found higher mtDNA content in 84% of their NAION subjects. The same authors previously reported an …

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