Transport of Proteins into Mitochondria

Mitochondrial protein import regulates cytosolic protein homeostasis and neuronal integrity

(2018) Wei Liu et al.   Autophagy

Vps39 Interacts with Tom40 to Establish One of Two Functionally Distinct Vacuole-Mitochondria Contact Sites

(2018) Ayelén González Montoro et al.   DEVELOPMENTAL CELL

Tom70 enhances mitochondrial preprotein import efficiency by binding to internal targeting sequences

(2018) Sandra Backes et al.   JOURNAL OF CELL BIOLOGY

Cytosolic Hsp70 and Hsp40 chaperones enable the biogenesis of mitochondrial β-barrel proteins

(2018) Tobias Jores et al.   JOURNAL OF CELL BIOLOGY

The GET pathway can increase the risk of mitochondrial outer membrane proteins to be mistargeted to the ER

(2018) Daniela G. Vitali et al.   JOURNAL OF CELL SCIENCE

Molecular and structural architecture of polyQ aggregates in yeast

(2018) Anselm Gruber et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Membrane protein insertion through a mitochondrial β-barrel gate

(2018) Alexandra I. C. Höhr et al.   SCIENCE

MitoCPR—A surveillance pathway that protects mitochondria in response to protein import stress

(2018) Hilla Weidberg et al.   SCIENCE

Defining the physiological role of SRP in protein-targeting efficiency and specificity

(2018) Elizabeth A. Costa et al.   SCIENCE

Mutations of the mitochondrial carrier translocase channel subunit TIM22 cause early-onset mitochondrial myopathy

(2018) David Pacheu-Grau et al.   HUMAN MOLECULAR GENETICS

The ribosome receptors Mrx15 and Mba1 jointly organize cotranslational insertion and protein biogenesis in mitochondria

(2018) Braulio Vargas Möller-Hergt et al.   MOLECULAR BIOLOGY OF THE CELL

Unique features of mammalian mitochondrial translation initiation revealed by cryo-EM

(2018) Eva Kummer et al.   NATURE

An ER surface retrieval pathway safeguards the import of mitochondrial membrane proteins in yeast

(2018) Katja G. Hansen et al.   SCIENCE

Pex19 is involved in importing dually targeted tail-anchored proteins to both mitochondria and peroxisomes

(2018) Bogdan A. Cichocki et al.   TRAFFIC

Structural Basis of Membrane Protein Chaperoning through the Mitochondrial Intermembrane Space

(2018) Katharina Weinhäupl et al.   CELL

Recruitment of Cytosolic J-Proteins by TOM Receptors Promotes Mitochondrial Protein Biogenesis

(2018) Łukasz Opaliński et al.   Cell Reports

αβ′-NAC cooperates with Sam37 to mediate early stages of mitochondrial protein import

(2017) José Carlos Ponce-Rojas et al.   FEBS Journal

Adaptation of a Genetic Screen Reveals an Inhibitor for Mitochondrial Protein Import Component Tim44

(2017) Non Miyata et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The MICOS component Mic60 displays a conserved membrane-bending activity that is necessary for normal cristae morphology

(2017) Daryna Tarasenko et al.   JOURNAL OF CELL BIOLOGY

Vps13-Mcp1 interact at vacuole–mitochondria interfaces and bypass ER–mitochondria contact sites

(2017) Arun T. John Peter et al.   JOURNAL OF CELL BIOLOGY

Structure–function insights into direct lipid transfer between membranes by Mmm1–Mdm12 of ERMES

(2017) Shin Kawano et al.   JOURNAL OF CELL BIOLOGY

Predicting the targeting of tail-anchored proteins to subcellular compartments in mammalian cells

(2017) Joseph L. Costello et al.   JOURNAL OF CELL SCIENCE

Targeting and translocation of proteins to the endoplasmic reticulum at a glance

(2017) Naama Aviram et al.   JOURNAL OF CELL SCIENCE

Sengers Syndrome-Associated Mitochondrial Acylglycerol Kinase Is a Subunit of the Human TIM22 Protein Import Complex

(2017) Yilin Kang et al.   MOLECULAR CELL

Acylglycerol Kinase Mutated in Sengers Syndrome Is a Subunit of the TIM22 Protein Translocase in Mitochondria

(2017) Milena Vukotic et al.   MOLECULAR CELL

Crystal structures of Mmm1 and Mdm12–Mmm1 reveal mechanistic insight into phospholipid trafficking at ER-mitochondria contact sites

(2017) Hanbin Jeong et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ER-mitochondria tethering by PDZD8 regulates Ca2+dynamics in mammalian neurons

(2017) Yusuke Hirabayashi et al.   SCIENCE

Regulated membrane remodeling by Mic60 controls formation of mitochondrial crista junctions

(2017) Manuel Hessenberger et al.   Nature Communications

Landscape of submitochondrial protein distribution

(2017) F.-Nora Vögtle et al.   Nature Communications

Definition of a High-Confidence Mitochondrial Proteome at Quantitative Scale

(2017) Marcel Morgenstern et al.   Cell Reports

Mitochondrial Stress Restores the Heat Shock Response and Prevents Proteostasis Collapse during Aging

(2017) Johnathan Labbadia et al.   Cell Reports

Dual interaction of scaffold protein Tim44 of mitochondrial import motor with channel-forming translocase subunit Tim23

(2017) See-Yeun Ting et al.   eLife

Role of Tim17 in coupling the import motor to the translocation channel of the mitochondrial presequence translocase

(2017) Keren Demishtein-Zohary et al.   eLife

Live-cell mapping of organelle-associated RNAs via proximity biotinylation combined with protein-RNA crosslinking

(2017) Pornchai Kaewsapsak et al.   eLife

Proteomic mapping of cytosol-facing outer mitochondrial and ER membranes in living human cells by proximity biotinylation

(2017) Victoria Hung et al.   eLife

The Transcription Factor ATF5 Mediates a Mammalian Mitochondrial UPR

(2016) Christopher J. Fiorese et al.   CURRENT BIOLOGY

Mcp3 is a novel mitochondrial outer membrane protein that follows a unique IMP‐dependent biogenesis pathway

(2016) Monika Sinzel et al.   EMBO REPORTS

The cytosolic cochaperone Sti1 is relevant for mitochondrial biogenesis and morphology

(2016) Hoda Hoseini et al.   FEBS Journal

TIM29 is a subunit of the human carrier translocase required for protein transport

(2016) Sylvie Callegari et al.   FEBS LETTERS

Heat Shock Protein 90 kDa (Hsp90) Has a Second Functional Interaction Site with the Mitochondrial Import Receptor Tom70

(2016) Leticia M. Zanphorlin et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Two distinct membrane potential–dependent steps drive mitochondrial matrix protein translocation

(2016) Alexander Benjamin Schendzielorz et al.   JOURNAL OF CELL BIOLOGY

A disulfide bond in the TIM23 complex is crucial for voltage gating and mitochondrial protein import

(2016) Ajay Ramesh et al.   JOURNAL OF CELL BIOLOGY

Ubiquilins Chaperone and Triage Mitochondrial Membrane Proteins for Degradation

(2016) Eisuke Itakura et al.   MOLECULAR CELL

The SND proteins constitute an alternative targeting route to the endoplasmic reticulum

(2016) Naama Aviram et al.   NATURE

Separating mitochondrial protein assembly and endoplasmic reticulum tethering by selective coupling of Mdm10

(2016) Lars Ellenrieder et al.   Nature Communications

Characterization of the targeting signal in mitochondrial β-barrel proteins

(2016) Tobias Jores et al.   Nature Communications

An Essential Role for COPI in mRNA Localization to Mitochondria and Mitochondrial Function

(2016) Dmitry Zabezhinsky et al.   Cell Reports

Mia40 is a trans-site receptor that drives protein import into the mitochondrial intermembrane space by hydrophobic substrate binding

(2016) Valentina Peleh et al.   eLife

Tim29 is a novel subunit of the human TIM22 translocase and is involved in complex assembly and stability

(2016) Yilin Kang et al.   eLife

A presequence-binding groove in Tom70 supports import of Mdl1 into mitochondria

(2015) Jonathan Melin et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Polyglutamine toxicity in yeast induces metabolic alterations and mitochondrial defects

(2015) Katharina Papsdorf et al.   BMC GENOMICS

Mic10 Oligomerizes to Bend Mitochondrial Inner Membranes at Cristae Junctions

(2015) Mariam Barbot et al.   Cell Metabolism

Central Role of Mic10 in the Mitochondrial Contact Site and Cristae Organizing System

(2015) Maria Bohnert et al.   Cell Metabolism

The Ca2+-Dependent Release of the Mia40-Induced MICU1-MICU2 Dimer from MCU Regulates Mitochondrial Ca2+ Uptake

(2015) Carmelina Petrungaro et al.   Cell Metabolism

GxxxG motifs hold the TIM23 complex together

(2015) Keren Demishtein-Zohary et al.   FEBS Journal

Conservation of Transit Peptide-Independent Protein Import into the Mitochondrial and Hydrogenosomal Matrix

(2015) Sriram Garg et al.   Genome Biology and Evolution

Cooperation of TOM and TIM23 Complexes during Translocation of Proteins into Mitochondria

(2015) Karin Waegemann et al.   JOURNAL OF MOLECULAR BIOLOGY

Quantitative Profiling for Substrates of the Mitochondrial Presequence Processing Protease Reveals a Set of Nonsubstrate Proteins Increased upon Proteotoxic Stress

(2015) Julia M. Burkhart et al.   JOURNAL OF PROTEOME RESEARCH

A cytosolic network suppressing mitochondria-mediated proteostatic stress and cell death

(2015) Xiaowen Wang et al.   NATURE

Mistargeted mitochondrial proteins activate a proteostatic response in the cytosol

(2015) Lidia Wrobel et al.   NATURE

MitoCarta2.0: an updated inventory of mammalian mitochondrial proteins

(2015) Sarah E. Calvo et al.   NUCLEIC ACIDS RESEARCH

The principle of antagonism ensures protein targeting specificity at the endoplasmic reticulum

(2015) M. Gamerdinger et al.   SCIENCE

Molecular architecture of the active mitochondrial protein gate

(2015) T. Shiota et al.   SCIENCE

Organization of the mitochondrial translation machinery studied in situ by cryoelectron tomography

(2015) Stefan Pfeffer et al.   Nature Communications

Mitochondrial protein import receptors in Kinetoplastids reveal convergent evolution over large phylogenetic distances

(2015) Jan Mani et al.   Nature Communications

Lam6 Regulates the Extent of Contacts between Organelles

(2015) Yael Elbaz-Alon et al.   Cell Reports

Protein translocation channel of mitochondrial inner membrane and matrix-exposed import motor communicate via two-domain coupling protein

(2015) Rupa Banerjee et al.   eLife

Msp1/ATAD1 maintains mitochondrial function by facilitating the degradation of mislocalized tail-anchored proteins

(2014) Y.-C. Chen et al.   EMBO JOURNAL

Presequence Recognition by the Tom40 Channel Contributes to Precursor Translocation into the Mitochondrial Matrix

(2014) J. Melin et al.   MOLECULAR AND CELLULAR BIOLOGY

Proteomic Mapping of the Human Mitochondrial Intermembrane Space in Live Cells via Ratiometric APEX Tagging

(2014) Victoria Hung et al.   MOLECULAR CELL

Mgr2 Functions as Lateral Gatekeeper for Preprotein Sorting in the Mitochondrial Inner Membrane

(2014) Raffaele Ieva et al.   MOLECULAR CELL

The conserved AAA-ATPase Msp1 confers organelle specificity to tail-anchored proteins

(2014) V. Okreglak et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Targeting and plasticity of mitochondrial proteins revealed by proximity-specific ribosome profiling

(2014) C. C. Williams et al.   SCIENCE

Principles of ER cotranslational translocation revealed by proximity-specific ribosome profiling

(2014) C. H. Jan et al.   SCIENCE

Cell cycle-dependent regulation of mitochondrial preprotein translocase

(2014) A. B. Harbauer et al.   SCIENCE

OM14 is a mitochondrial receptor for cytosolic ribosomes that supports co-translational import into mitochondria

(2014) Chen Lesnik et al.   Nature Communications

Aim24 and MICOS modulate respiratory function, tafazzin-related cardiolipin modification and mitochondrial architecture

(2014) Max Emanuel Harner et al.   eLife

New insights into the targeting of a subset of tail-anchored proteins to the outer mitochondrial membrane

(2014) Naomi J. Marty et al.   Frontiers in Plant Science

Mdm10 is an ancient eukaryotic porin co-occurring with the ERMES complex

(2013) Nadine Flinner et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Signal recognition initiates reorganization of the presequence translocase during protein import

(2013) Oleksandr Lytovchenko et al.   EMBO JOURNAL

Mia40 and MINOS act in parallel with Ccs1 in the biogenesis of mitochondrial Sod1

(2013) Aksana Varabyova et al.   FEBS Journal

The Role of Djp1 in Import of the Mitochondrial Protein Mim1 Demonstrates Specificity between a Cochaperone and Its Substrate Protein

(2013) D. Papic et al.   MOLECULAR AND CELLULAR BIOLOGY

Sequential Duplications of an Ancient Member of the DnaJ-Family Expanded the Functional Chaperone Network in the Eukaryotic Cytosol

(2013) Chandan Sahi et al.   MOLECULAR BIOLOGY AND EVOLUTION

Protein import and oxidative folding in the mitochondrial intermembrane space of intact mammalian cells

(2013) Manuel Fischer et al.   MOLECULAR BIOLOGY OF THE CELL

Evidence of Evolutionary Constraints That Influences the Sequence Composition and Diversity of Mitochondrial Matrix Targeting Signals

(2013) Stephen R. Doyle et al.   PLoS One

Atp23 biogenesis reveals a chaperone-like folding activity of Mia40 in the IMS of mitochondria

(2012) Daniel Weckbecker et al.   EMBO JOURNAL

Structural characterization of CHCHD5 and CHCHD7: Two atypical human twin CX9C proteins

(2012) Lucia Banci et al.   JOURNAL OF STRUCTURAL BIOLOGY

Intermembrane Space Proteome of Yeast Mitochondria

(2012) F.-Nora Vögtle et al.   MOLECULAR & CELLULAR PROTEOMICS

The C-terminal domain of Fcj1 is required for formation of crista junctions and interacts with the TOB/SAM complex in mitochondria

(2012) Christian Körner et al.   MOLECULAR BIOLOGY OF THE CELL

Ergosterol content specifies targeting of tail-anchored proteins to mitochondrial outer membranes

(2012) Katrin Krumpe et al.   MOLECULAR BIOLOGY OF THE CELL

Eukaryote-wide sequence analysis of mitochondrial β-barrel outer membrane proteins

(2011) Kenichiro Imai et al.   BMC GENOMICS

Regulation of Mitochondrial Protein Import by Cytosolic Kinases

(2011) Oliver Schmidt et al.   CELL

MINOS Is Plus: A Mitofilin Complex for Mitochondrial Membrane Contacts

(2011) Johannes M. Herrmann   DEVELOPMENTAL CELL

Dual Role of Mitofilin in Mitochondrial Membrane Organization and Protein Biogenesis

(2011) Karina von der Malsburg et al.   DEVELOPMENTAL CELL

Interaction between the Human Mitochondrial Import Receptors Tom20 and Tom70in VitroSuggests a Chaperone Displacement Mechanism

(2011) Anna C. Y. Fan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mia40-dependent oxidation of cysteines in domain I of Ccs1 controls its distribution between mitochondria and the cytosol

(2011) Christine Klöppel et al.   MOLECULAR BIOLOGY OF THE CELL

Dual Function of Sdh3 in the Respiratory Chain and TIM22 Protein Translocase of the Mitochondrial Inner Membrane

(2011) Natalia Gebert et al.   MOLECULAR CELL

Nanoscale distribution of mitochondrial import receptor Tom20 is adjusted to cellular conditions and exhibits an inner-cellular gradient

(2011) C. A. Wurm et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

In vivo protein-interaction mapping of a mitochondrial translocator protein Tom22 at work

(2011) T. Shiota et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Localization of mRNAs coding for mitochondrial proteins in the yeast Saccharomyces cerevisiae

(2011) N. Gadir et al.   RNA

The Intermembrane Space of Mitochondria

(2010) Johannes M. Herrmann et al.   ANTIOXIDANTS & REDOX SIGNALING

Roles of the Mdm10, Tom7, Mdm12, and Mmm1 Proteins in the Assembly of Mitochondrial Outer Membrane Proteins in Neurospora crassa

(2010) Jeremy G. Wideman et al.   MOLECULAR BIOLOGY OF THE CELL

Assembly of the Mitochondrial Protein Import Channel

(2010) Thomas Becker et al.   MOLECULAR BIOLOGY OF THE CELL

Dual role of the receptor Tom20 in specificity and efficiency of protein import into mitochondria

(2010) H. Yamamoto et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Substrate specificity of the TIM22 mitochondrial import pathway revealed with small molecule inhibitor of protein translocation

(2010) S. A. Hasson et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Global Analysis of the Mitochondrial N-Proteome Identifies a Processing Peptidase Critical for Protein Stability

(2009) F.-Nora Vögtle et al.   CELL

Importing Mitochondrial Proteins: Machineries and Mechanisms

(2009) Agnieszka Chacinska et al.   CELL

Roles of Tom70 in Import of Presequence-containing Mitochondrial Proteins

(2009) Hayashi Yamamoto et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A novel intermembrane space–targeting signal docks cysteines onto Mia40 during mitochondrial oxidative folding

(2009) Dionisia P. Sideris et al.   JOURNAL OF CELL BIOLOGY

Systematic Analysis of the Twin Cx9C Protein Family

(2009) Sebastian Longen et al.   JOURNAL OF MOLECULAR BIOLOGY

Tom20 Mediates Localization of mRNAs to Mitochondria in a Translation-Dependent Manner

(2009) E. Eliyahu et al.   MOLECULAR AND CELLULAR BIOLOGY

Identification of the Signal Directing Tim9 and Tim10 into the Intermembrane Space of Mitochondria

(2009) Dusanka Milenkovic et al.   MOLECULAR BIOLOGY OF THE CELL

Refining the Definition of Plant Mitochondrial Presequences through Analysis of Sorting Signals, N-Terminal Modifications, and Cleavage Motifs

(2009) S. Huang et al.   PLANT PHYSIOLOGY

An ER-Mitochondria Tethering Complex Revealed by a Synthetic Biology Screen

(2009) Benoît Kornmann et al.   SCIENCE

The GET Complex Mediates Insertion of Tail-Anchored Proteins into the ER Membrane

(2008) Maya Schuldiner et al.   CELL

A Molecular Switch for Targeting between Endoplasmic Reticulum (ER) and Mitochondria

(2008) Yuliang Ma et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Structural and Functional Roles of the Conserved Cysteine Residues of the Redox-regulated Import Receptor Mia40 in the Intermembrane Space of Mitochondria

(2008) Nadia Terziyska et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Integration of tail-anchored proteins into the mitochondrial outer membrane does not require any known import components

(2008) C. Kemper et al.   JOURNAL OF CELL SCIENCE

Cryo-Electron Microscopy Structure of a Yeast Mitochondrial Preprotein Translocase

(2008) Kirstin Model et al.   JOURNAL OF MOLECULAR BIOLOGY

Structural and Functional Requirements for Activity of the Tim9–Tim10 Complex in Mitochondrial Protein Import

(2008) Michael J. Baker et al.   MOLECULAR BIOLOGY OF THE CELL

Yeast Mitochondrial Biogenesis: A Role for the PUF RNA-Binding Protein Puf3p in mRNA Localization

(2008) Yann Saint-Georges et al.   PLoS One