Mitochondria play an essential role in synthesis and export of iron–sulfur (Fe–S) clusters to other sections of a cell. Although the mechanism of Fe–S cluster synthesis is well elucidated, information on the identity of the proteins involved in the export pathway is limited. The present study identifies hMIA40 (human mitochondrial intermembrane space import and assembly protein 40), also known as CHCHD4 (coiled-coil–helix–coiled-coil–helix domain-containing 4), as a component of the mitochondrial Fe–S cluster export machinery. hMIA40 is an iron-binding protein with the ability to bind iron in vivo and in vitro. hMIA40 harbours CPC (Cys-Pro-Cys) motif-dependent Fe–S clusters that are sensitive to oxidation. Depletion of hMIA40 results in accumulation of iron in mitochondria concomitant with decreases in the activity and stability of Fe–S-containing cytosolic enzymes. Intriguingly, overexpression of either the mitochondrial export component or cytosolic the Fe–S cluster assembly component does not have any effect on the phenotype of hMIA40-depleted cells. Taken together, our results demonstrate an indispensable role for hMIA40 for the export of Fe–S clusters from mitochondria.
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Research Article|
October 02 2015
Human mitochondrial MIA40 (CHCHD4) is a component of the Fe–S cluster export machinery
Anjaneyulu Murari;
Anjaneyulu Murari
*Department of Biochemistry, University of Hyderabad, Gachibowli, Hyderabad 500046, India
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Venkata Ramana Thiriveedi;
Venkata Ramana Thiriveedi
*Department of Biochemistry, University of Hyderabad, Gachibowli, Hyderabad 500046, India
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Fareed Mohammad;
Fareed Mohammad
*Department of Biochemistry, University of Hyderabad, Gachibowli, Hyderabad 500046, India
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Viswamithra Vengaldas;
Viswamithra Vengaldas
*Department of Biochemistry, University of Hyderabad, Gachibowli, Hyderabad 500046, India
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Madhavi Gorla;
Madhavi Gorla
*Department of Biochemistry, University of Hyderabad, Gachibowli, Hyderabad 500046, India
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Prasad Tammineni;
Prasad Tammineni
*Department of Biochemistry, University of Hyderabad, Gachibowli, Hyderabad 500046, India
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Thanuja Krishnamoorthy;
Thanuja Krishnamoorthy
*Department of Biochemistry, University of Hyderabad, Gachibowli, Hyderabad 500046, India
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Naresh Babu V. Sepuri
Naresh Babu V. Sepuri
1
*Department of Biochemistry, University of Hyderabad, Gachibowli, Hyderabad 500046, India
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Publisher: Portland Press Ltd
Received:
January 05 2015
Revision Received:
August 05 2015
Accepted:
August 14 2015
Accepted Manuscript online:
August 14 2015
Online ISSN: 1470-8728
Print ISSN: 0264-6021
© 2015 Authors; published by Portland Press Limited
2015
Biochem J (2015) 471 (2): 231–241.
Article history
Received:
January 05 2015
Revision Received:
August 05 2015
Accepted:
August 14 2015
Accepted Manuscript online:
August 14 2015
Citation
Anjaneyulu Murari, Venkata Ramana Thiriveedi, Fareed Mohammad, Viswamithra Vengaldas, Madhavi Gorla, Prasad Tammineni, Thanuja Krishnamoorthy, Naresh Babu V. Sepuri; Human mitochondrial MIA40 (CHCHD4) is a component of the Fe–S cluster export machinery. Biochem J 15 October 2015; 471 (2): 231–241. doi: https://doi.org/10.1042/BJ20150012
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