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Mitochondrial complex I and cell death: a semi-automatic shotgun model.

ABSTRACT: Mitochondrial dysfunction often leads to cell death and disease. We can now draw correlations between the dysfunction of one of the most important mitochondrial enzymes, NADH:ubiquinone reductase or complex I, and its structural organization thanks to the recent advances in the X-ray structure of its bacterial homologs. The new structural information on bacterial complex I provide essential clues to finally understand how complex I may work. However, the same information remains difficult to interpret for many scientists working on mitochondrial complex I from different angles, especially in the field of cell death. Here, we present a novel way of interpreting the bacterial structural information in accessible terms. On the basis of the analogy to semi-automatic shotguns, we propose a novel functional model that incorporates recent structural information with previous evidence derived from studies on mitochondrial diseases, as well as functional bioenergetics.

SUBMITTER: Gonzalez-Halphen D

PROVIDER: S-EPMC3219089 | biostudies-other | 2011 Oct

REPOSITORIES: biostudies-other

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Mitochondrial complex I and cell death: a semi-automatic shotgun model.

Gonzalez-Halphen D D Ghelli A A Iommarini L L Carelli V V Esposti M D MD

Cell death & disease 20111027

Mitochondrial dysfunction often leads to cell death and disease. We can now draw correlations between the dysfunction of one of the most important mitochondrial enzymes, NADH:ubiquinone reductase or complex I, and its structural organization thanks to the recent advances in the X-ray structure of its bacterial homologs. The new structural information on bacterial complex I provide essential clues to finally understand how complex I may work. However, the same information remains difficult to int ...[more]

PMID: 22030538

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