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

Angelika B. Harbauer, René P. Zahedi, Albert Sickmann, Nikolaus Pfanner, Chris Meisinger

Research output: Contribution to journalReview articlepeer-review

290 Scopus citations

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.

Original languageEnglish
Pages (from-to)357-372
Number of pages16
JournalCell Metabolism
Volume19
Issue number3
DOIs
StatePublished - 4 Mar 2014
Externally publishedYes

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