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Comment
. 2023 Nov 7;120(45):e2315649120.
doi: 10.1073/pnas.2315649120. Epub 2023 Oct 25.

How defective mitochondrial electrical activity leads to inherited blindness

Peter J Burke  1   2   3   4
Affiliations
Comment

How defective mitochondrial electrical activity leads to inherited blindness

Peter J Burke. Proc Natl Acad Sci U S A. .
No abstract available

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Conflict of interest statement

The author has filed patents related to microfluidic mitochondrial assays through his employment at the University of California, Irvine. The author is in the process of founding a company related to microfluidic mitochondrial assays.

Figures

Fig. 1.
Fig. 1.
(A) Schematic and functional diagram of the electron transport chain complexes I–V on the mitochondrial inner membrane. (B) Structural and functional detail of complex I, showing the LHON mutation site and its relationship to the path electrons traverse from the initial oxidation of NADH by CI to the final reduction of CoQ10 by CI. (C) Cartoon analog of the finding of Fuller et al. (4). A and B adopted from refs. and , with permission.
See this image and copyright information in PMC

Comment on

References

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    1. Wallace D. C., Lott M. T., Procaccio V., “Mitochondrial biology and medicine” in Emery and Rimoin’s Principles and Practice of Medical Genetics and Genomics: Foundations, (Elsevier, 2018), pp. 267–322.
    1. Carelli V., Ross-Cisneros F. N., Sadun A. A., Mitochondrial dysfunction as a cause of optic neuropathies. Prog. Retin. Eye Res. 23, 53–89 (2004). - PubMed
    1. Fuller J. T., et al. , Coenzyme Q10 trapping in mitochondrial Complex I underlies Leber’s hereditary optic neuropathy. Proc. Natl. Acad. Sci. U.S.A. 120, e2304884120 (2023). - PMC - PubMed
    1. Gutiérrez-Fernández J., et al. , Key role of quinone in the mechanism of respiratory complex I. Nat. Commun. 11, 4135 (2020). - PMC - PubMed

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