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Open AccessJournal ArticleDOI

Mitochondrial protein import: common principles and physiological networks

TLDR
Recent studies that indicate how the pathways for mitochondrial protein biogenesis are embedded into a functional network of various other physiological processes, such as energy metabolism, signal transduction, and maintenance of mitochondrial morphology are discussed.
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This article is published in Biochimica et Biophysica Acta.The article was published on 2013-02-01 and is currently open access. It has received 257 citations till now. The article focuses on the topics: mitochondrial fusion & Mitochondrial fission.

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A conserved endoplasmic reticulum membrane protein complex (EMC) facilitates phospholipid transfer from the ER to mitochondria.

TL;DR: Tethering of the endoplasmic reticulum to mitochondria by a conserved endoplasmsic Reticulum complex is needed for the transfer of phospholipids between these organelles.
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Role of Cardiolipin in Mitochondrial Signaling Pathways.

TL;DR: The role of CL in cellular signaling pathways is summarized and focus on tissues with high-energy demand, like the heart, and the signaling pathways described here may play a general role in pathology.
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Eukaryotic 5-methylcytosine (m5C) RNA Methyltransferases: Mechanisms, Cellular Functions, and Links to Disease

TL;DR: Functional characterizations of these proteins and the modifications they install have revealed important roles in diverse aspects of both mitochondrial and nuclear gene expression, and enabled a better understanding of the molecular basis of a number of diseases caused by mutations in the genes encoding m5C methyltransferases or changes in the expression level of these enzymes.
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Diversity and selectivity in mRNA translation on the endoplasmic reticulum

TL;DR: The authors showed that ER-bound ribosomes also function in the translation of a large fraction of mRNAs that encode cytosolic proteins, suggesting a far more expansive role for the ER in transcriptome expression, where membrane and secretory protein synthesis represents one element of a multifaceted and dynamic contribution to posttranscriptional gene expression.
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Chemistry and Enzymology of Disulfide Cross-Linking in Proteins.

TL;DR: This work lays the groundwork for appreciating the mechanisms and consequences of disulfide bond formation in vivo by reviewing chemical principles underlying cysteine pairing and oxidation, and shows how enzymes tune redox-active cofactors and recruit oxidants to improve the specificity and efficiency ofdisulfide formation.
References
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Journal ArticleDOI

A Mitochondrial Protein Compendium Elucidates Complex I Disease Biology

TL;DR: This work predicts 19 proteins to be important for the function of complex I (CI) of the electron transport chain and validate a subset of these predictions using RNAi, including C8orf38, which is shown to have an inherited mutation in a lethal, infantile CI deficiency.
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Translocation of proteins into mitochondria.

TL;DR: This review summarizes the present knowledge on the import and sorting of mitochondrial precursor proteins, with a special emphasis on unresolved questions and topics of current research.
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Supercomplexes in the respiratory chains of yeast and mammalian mitochondria

TL;DR: A fraction of total bovine ATP synthase was isolated in dimeric form, suggesting that a dimeric state is not limited to S.cerevisiae, but also exists in mammalian mitochondria, which guided us to present a model for a network of respiratory chain complexes: a ‘respirasome’.
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An ER-Mitochondria Tethering Complex Revealed by a Synthetic Biology Screen

TL;DR: In this article, the authors identified the Mmm1/Mdm10/mdm12/mdr34 complex as a molecular tether between the endoplasmic reticulum (ER) and mitochondria.
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The proteome of Saccharomyces cerevisiae mitochondria

TL;DR: The mitochondrial proteome will provide an important database for the analysis of new mitochondrial and mitochondria-associated functions and the characterization of mitochondrial diseases.
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