Mitochondrial protein import clogging as a mechanism of disease

MitoStores: chaperone‐controlled protein granules store mitochondrial precursors in the cytosol

(2023) Lena Krämer et al.   EMBO JOURNAL

Functional crosstalk between the TIM22 complex and YME1 machinery maintains mitochondrial proteostasis and integrity

(2023) Abhishek Kumar et al.   JOURNAL OF CELL SCIENCE

Mitochondrial complexome reveals quality-control pathways of protein import

(2023) Uwe Schulte et al.   NATURE

Crosstalk between Mitochondrial Protein Import and Lipids

(2022) Juliane J. Hoffmann et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

The UPRmt preserves mitochondrial import to extend lifespan

(2022) Nan Xin et al.   JOURNAL OF CELL BIOLOGY

Mistargeting of aggregation prone mitochondrial proteins activates a nucleus-mediated posttranscriptional quality control pathway in trypanosomes

(2022) Caroline E. Dewar et al.   Nature Communications

ER targeting of non-imported mitochondrial carrier proteins is dependent on the GET pathway

(2021) Tianyao Xiao et al.   Life Science Alliance

Impaired phosphatidylethanolamine metabolism activates a reversible stress response that detects and resolves mutant mitochondrial precursors

(2021) Pingdewinde N. Sam et al.   iScience

The chaperone-binding activity of the mitochondrial surface receptor Tom70 protects the cytosol against mitoprotein-induced stress

(2021) Sandra Backes et al.   Cell Reports

A nuclear-based quality control pathway for non-imported mitochondrial proteins

(2021) Viplendra PS Shakya et al.   eLife

Mitochondrial DNA depletion syndrome with a mutation in SLC25A4 developing epileptic encephalopathy: A case report

(2021) Tomoko Kashiki et al.   BRAIN & DEVELOPMENT

Quantitative high-confidence human mitochondrial proteome and its dynamics in cellular context

(2021) Marcel Morgenstern et al.   Cell Metabolism

Mutations in TOMM70 lead to multi-OXPHOS deficiencies and cause severe anemia, lactic acidosis, and developmental delay

(2020) Xiujuan Wei et al.   JOURNAL OF HUMAN GENETICS

De novo mutations in TOMM70, a receptor of the mitochondrial import translocase, cause neurological impairment

(2020) Debdeep Dutta et al.   HUMAN MOLECULAR GENETICS

Dynamic Regulation of Mitochondrial Import by the Ubiquitin System

(2020) Lilian Phu et al.   MOLECULAR CELL

Global Landscape and Dynamics of Parkin and USP30-Dependent Ubiquitylomes in iNeurons during Mitophagic Signaling

(2020) Alban Ordureau et al.   MOLECULAR CELL

MetaboAnalystR 3.0: Toward an Optimized Workflow for Global Metabolomics

(2020) Zhiqiang Pang et al.   Metabolites

The mitochondrial ADP/ATP carrier exists and functions as a monomer

(2020) Edmund R.S. Kunji et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Structure of the mitochondrial TIM22 complex from yeast

(2020) Yutong Zhang et al.   CELL RESEARCH

Cryo-EM structure of the human mitochondrial translocase TIM22 complex

(2020) Liangbo Qi et al.   CELL RESEARCH

Quality control of the mitochondrial proteome

(2020) Jiyao Song et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

The Molecular Mechanism of Transport by the Mitochondrial ADP/ATP Carrier

(2019) Jonathan J. Ruprecht et al.   CELL

Mitochondrial proteins: from biogenesis to functional networks

(2019) Nikolaus Pfanner et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Dual Role of Mitochondrial Porin in Metabolite Transport across the Outer Membrane and Protein Transfer to the Inner Membrane

(2019) Lars Ellenrieder et al.   MOLECULAR CELL

Mitochondrial carrier protein overloading and misfolding induce aggresomes and proteostatic adaptations in the cytosol

(2019) Yaxin Liu et al.   MOLECULAR BIOLOGY OF THE CELL

Mitochondrial protein translocation-associated degradation

(2019) Christoph U. Mårtensson et al.   NATURE

Publisher Correction: Mitochondrial protein-induced stress triggers a global adaptive transcriptional programme

(2019) Felix Boos et al.   NATURE CELL BIOLOGY

Structure and function of Vms1 and Arb1 in RQC and mitochondrial proteome homeostasis

(2019) Ting Su et al.   NATURE

Consequences of inner mitochondrial membrane protein misfolding

(2019) Liam P. Coyne et al.   MITOCHONDRION

Structure of the mitochondrial import gate reveals distinct preprotein paths

(2019) Yuhei Araiso et al.   NATURE

Two new cases of mitochondrial myopathy with exercise intolerance, hyperlactatemia and cardiomyopathy, caused by recessive SLC25A4 mutations

(2018) Anouk Tosserams et al.   MITOCHONDRION

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

(2018) Hilla Weidberg et al.   SCIENCE

Vms1p is a release factor for the ribosome-associated quality control complex

(2018) Olga Zurita Rendón et al.   Nature Communications

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

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

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

(2018) Katja G. Hansen et al.   SCIENCE

Structural Basis of Membrane Protein Chaperoning through the Mitochondrial Intermembrane Space

(2018) Katharina Weinhäupl et al.   CELL

STRING v11: protein–protein association networks with increased coverage, supporting functional discovery in genome-wide experimental datasets

(2018) Damian Szklarczyk et al.   NUCLEIC ACIDS RESEARCH

mPOS is a novel mitochondrial trigger of cell death - implications for neurodegeneration

(2017) Liam P. Coyne et al.   FEBS LETTERS

Mitochondrial inner-membrane protease Yme1 degrades outer-membrane proteins Tom22 and Om45

(2017) Xi Wu et al.   JOURNAL OF CELL BIOLOGY

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

Mitochondrial energy deficiency leads to hyperproliferation of skeletal muscle mitochondria and enhanced insulin sensitivity

(2017) Ryan M. Morrow et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Definition of a High-Confidence Mitochondrial Proteome at Quantitative Scale

(2017) Marcel Morgenstern et al.   Cell Reports

Recurrent De Novo Dominant Mutations in SLC25A4 Cause Severe Early-Onset Mitochondrial Disease and Loss of Mitochondrial DNA Copy Number

(2016) Kyle Thompson et al.   AMERICAN JOURNAL OF HUMAN GENETICS

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

(2016) Sylvie Callegari et al.   FEBS LETTERS

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

(2016) Yilin Kang et al.   eLife

Misfolding of mutant adenine nucleotide translocase in yeast supports a novel mechanism of Ant1-induced muscle diseases

(2015) Yaxin Liu et al.   MOLECULAR BIOLOGY OF THE CELL

Mitochondrial and Nuclear Accumulation of the Transcription Factor ATFS-1 Promotes OXPHOS Recovery during the UPRmt

(2015) Amrita M. Nargund et al.   MOLECULAR CELL

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

Molecular architecture of the active mitochondrial protein gate

(2015) T. Shiota et al.   SCIENCE

The c10orf10 gene product is a new link between oxidative stress and autophagy

(2014) Marcus W. Stepp et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Role of Phosphatidylethanolamine in the Biogenesis of Mitochondrial Outer Membrane Proteins

(2013) Thomas Becker et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Lack of the Mitochondrial Protein Acylglycerol Kinase Causes Sengers Syndrome

(2012) Johannes A. Mayr et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Roles of Dom34:Hbs1 in Nonstop Protein Clearance from Translocators for Normal Organelle Protein Influx

(2012) Toshiaki Izawa et al.   Cell Reports

Assessing mitochondrial dysfunction in cells

(2011) Martin D. Brand et al.   BIOCHEMICAL JOURNAL

Complete loss of expression of theANT1gene causing cardiomyopathy and myopathy

(2011) Andoni Echaniz-Laguna et al.   JOURNAL OF MEDICAL GENETICS

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

(2011) Natalia Gebert et al.   MOLECULAR CELL

Evolution, structure and function of mitochondrial carriers: a review with new insights

(2011) Ferdinando Palmieri et al.   PLANT JOURNAL

Mitochondrial Isolation from Skeletal Muscle

(2011) Mary L. Garcia-Cazarin et al.   Jove-Journal of Visualized Experiments

Skyline: an open source document editor for creating and analyzing targeted proteomics experiments

(2010) Brendan MacLean et al.   BIOINFORMATICS

The ribonucleotide reductase inhibitor, Sml1, is sequentially phosphorylated, ubiquitylated and degraded in response to DNA damage

(2010) Bethany L. Andreson et al.   NUCLEIC ACIDS RESEARCH

Multiple pathways for mitochondrial protein traffic

(2009) Toshiya Endo et al.   BIOLOGICAL CHEMISTRY

Validation of reference genes for quantitative expression analysis by real-time RT-PCR in Saccharomyces cerevisiae

(2009) Marie-Ange Teste et al.   BMC MOLECULAR BIOLOGY

Importing Mitochondrial Proteins: Machineries and Mechanisms

(2009) Agnieszka Chacinska et al.   CELL

Mitochondrial Cardiolipin Involved in Outer-Membrane Protein Biogenesis: Implications for Barth Syndrome

(2009) Natalia Gebert et al.   CURRENT BIOLOGY

Universal sample preparation method for proteome analysis

(2009) Jacek R Wiśniewski et al.   NATURE METHODS

A Mitochondrial Protein Compendium Elucidates Complex I Disease Biology

(2008) David J. Pagliarini et al.   CELL

Dominant membrane uncoupling by mutant adenine nucleotide translocase in mitochondrial diseases

(2008) X. Wang et al.   HUMAN MOLECULAR GENETICS

The Assembly Pathway of the Mitochondrial Carrier Translocase Involves Four Preprotein Translocases

(2008) K. Wagner et al.   MOLECULAR AND CELLULAR BIOLOGY

The Yeast Aac2 Protein Exists in Physical Association with the Cytochrome bc1-COX Supercomplex and the TIM23 Machinery

(2008) Mary K. Dienhart et al.   MOLECULAR BIOLOGY OF THE CELL