articleJournal of Biological ChemistryFeb 28, 2003HYBRID OA

Loss of OPA1 Perturbates the Mitochondrial Inner Membrane Structure and Integrity, Leading to Cytochrome c Release and Apoptosis

AOAurélien OlichonLBLaurent BaricaultNGNicole GasEGEmmanuelle GuillouAVAnnie Valette

Université Toulouse III - Paul Sabatier · Laboratoire de Biologie Moléculaire des Eucaryotes · +1 more institution

PubMed
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Abstract

OPA1 encodes a large GTPase related to dynamins, anchored to the mitochondrial cristae inner membrane, facing the intermembrane space. OPA1haplo-insufficiency is responsible for the most common form of autosomal dominant optic atrophy (ADOA, MIM165500), a neuropathy resulting from degeneration of the retinal ganglion cells and optic nerve atrophy. Here we show that down-regulation of OPA1 in HeLa cells using specific small interfering RNA (siRNA) leads to fragmentation of the mitochondrial network concomitantly to the dissipation of the mitochondrial membrane potential and to a drastic disorganization of the cristae. These events are followed by cytochromec release and caspase-dependent apoptotic nuclear…

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Topics & keywords

Topics
Keywords
  • Cell biology
  • Intermembrane space
  • Biology
  • Mitochondrion
  • Inner mitochondrial membrane
  • Apoptosis
  • Atrophy
  • Mitochondrial apoptosis-induced channel
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