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Comment
. 2009 Nov;1(8-9):357-9.
doi: 10.1002/emmm.200900049.

Making the most of what you've got: optimizing residual OXPHOS function in mitochondrial diseases

Carlos T Moraes  1
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Comment

Making the most of what you've got: optimizing residual OXPHOS function in mitochondrial diseases

Carlos T Moraes. EMBO Mol Med. 2009 Nov.

Abstract

Patients affected by mitochondrial OXPHOS disorders are still faced with a grim lack of therapeutic options. In this Closeup, Carlos Moraes revisits the recent data by Giovanni Manfredi on PKA's functions in the mitochondria and now its modulation can improve respiration and ATP production in COX-defective cells.

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Figures

Figure 1
Figure 1. Compensatory mechanisms for COX deficiencies
Acin Perez et al (2009) proposed that COX deficiencies can lead to a mitochondrial proliferation, possibly because of ROS signalling increasing PGC-1α expression. However, if increased activation by mt-sAC/PKA phosphorylation ensues, the levels of ROS are kept low and mitochondrial proliferation does not take place. The precise role of ROS in these pathways is still unclear.
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References

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