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Cell Metab. 2014 Mar 4;19(3):357-72. doi: 10.1016/j.cmet.2014.01.010. Epub 2014 Feb 20.

The protein import machinery of mitochondria-a regulatory hub in metabolism, stress, and disease.

Author information

1
Institut für Biochemie und Molekularbiologie, ZBMZ, Universität Freiburg, 79104 Freiburg, Germany; Trinationales Graduiertenkolleg 1478, Universität Freiburg, 79104 Freiburg, Germany; Faculty of Biology, Universität Freiburg, 79104 Freiburg, Germany; BIOSS Centre for Biological Signalling Studies, Universität Freiburg, 79104 Freiburg, Germany.
2
Leibniz-Institute for Analytical Sciences-ISAS-e.V., 44139 Dortmund, Germany.
3
Leibniz-Institute for Analytical Sciences-ISAS-e.V., 44139 Dortmund, Germany; Medizinisches Proteom-Center, Ruhr-Universität Bochum, 44801 Bochum, Germany.
4
Institut für Biochemie und Molekularbiologie, ZBMZ, Universität Freiburg, 79104 Freiburg, Germany; BIOSS Centre for Biological Signalling Studies, Universität Freiburg, 79104 Freiburg, Germany. Electronic address: nikolaus.pfanner@biochemie.uni-freiburg.de.
5
Institut für Biochemie und Molekularbiologie, ZBMZ, Universität Freiburg, 79104 Freiburg, Germany; BIOSS Centre for Biological Signalling Studies, Universität Freiburg, 79104 Freiburg, Germany. Electronic address: chris.meisinger@biochemie.uni-freiburg.de.

Abstract

Mitochondria fulfill central functions in bioenergetics, metabolism, and apoptosis. They import more than 1,000 different proteins from the cytosol. It had been assumed that the protein import machinery is constitutively active and not subject to detailed regulation. However, recent studies indicate that mitochondrial protein import is regulated at multiple levels connected to cellular metabolism, signaling, stress, and pathogenesis of diseases. Here, we discuss the molecular mechanisms of import regulation and their implications for mitochondrial homeostasis. The protein import activity can function as a sensor of mitochondrial fitness and provides a direct means of regulating biogenesis, composition, and turnover of the organelle.

PMID:
24561263
DOI:
10.1016/j.cmet.2014.01.010
[Indexed for MEDLINE]
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