The ‘mitochondrial contact site and cristae organising system’ (MICOS) in health and human disease

COmplexome Profiling ALignment (COPAL) reveals remodeling of mitochondrial protein complexes in Barth syndrome

(2019) Joeri Van Strien et al.   BIOINFORMATICS

Targeted OMA1 therapies for cancer

(2019) Marcel V. Alavi   INTERNATIONAL JOURNAL OF CANCER

Expanding and Underscoring the Hepato‐Encephalopathic Phenotype of QIL 1 / MIC 13

(2019) Bianca E Russell et al.   HEPATOLOGY

ALS/FTD mutant CHCHD10 mice reveal a tissue-specific toxic gain-of-function and mitochondrial stress response

(2019) Corey J. Anderson et al.   ACTA NEUROPATHOLOGICA

Mitochondrial defect in muscle precedes neuromuscular junction degeneration and motor neuron death in CHCHD10S59L/+ mouse

(2019) Emmanuelle C. Genin et al.   ACTA NEUROPATHOLOGICA

Sam50–Mic19–Mic60 axis determines mitochondrial cristae architecture by mediating mitochondrial outer and inner membrane contact

(2019) Junhui Tang et al.   CELL DEATH AND DIFFERENTIATION

Pyridostigmine alleviates cardiac dysfunction via improving mitochondrial cristae shape in a mouse model of metabolic syndrome

(2019) Run-Qing Xue et al.   FREE RADICAL BIOLOGY AND MEDICINE

A MICOS-TIM22 Association Promotes Carrier Import into Human Mitochondria

(2019) Sylvie Callegari et al.   JOURNAL OF MOLECULAR BIOLOGY

ER and Nutrient Stress Promote Assembly of Respiratory Chain Supercomplexes through the PERK-eIF2α Axis

(2019) Eduardo Balsa et al.   MOLECULAR CELL

Proteome‐based classification of Nonmuscle Invasive Bladder Cancer

(2019) Rafael Stroggilos et al.   INTERNATIONAL JOURNAL OF CANCER

Proteomic investigation of human skeletal muscle before and after 70 days of head down bed rest with or without exercise and testosterone countermeasures

(2019) E. Lichar Dillon et al.   PLoS One

PD-1 signaling affects cristae morphology and leads to mitochondrial dysfunction in human CD8+ T lymphocytes

(2019) Jesús Ogando et al.   Journal for ImmunoTherapy of Cancer

Individual cristae within the same mitochondrion display different membrane potentials and are functionally independent

(2019) Dane M Wolf et al.   EMBO JOURNAL

Armadillo repeat-containing protein 1 is a dual localization protein associated with mitochondrial intermembrane space bridging complex

(2019) Fabienne Wagner et al.   PLoS One

Miro clusters regulate ER-mitochondria contact sites and link cristae organization to the mitochondrial transport machinery

(2019) Souvik Modi et al.   Nature Communications

Mitochondrial inner membrane protein (mitofilin) knockdown induces cell death by apoptosis via an AIF-PARP-dependent mechanism and cell cycle arrest

(2018) Ngonidzashe B. Madungwe et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Diabetic Cardiomyopathy

(2018) Guanghong Jia et al.   CIRCULATION RESEARCH

CHCHD10 mutations p.R15L and p.G66V cause motoneuron disease by haploinsufficiency

(2018) Sarah J Brockmann et al.   HUMAN MOLECULAR GENETICS

ATAD3 controls mitochondrial cristae structure in mouse muscle, influencing mtDNA replication and cholesterol levels

(2018) Susana Peralta et al.   JOURNAL OF CELL SCIENCE

QIL1-dependent assembly of MICOS complex–lethal mutation in C19ORF70 resulting in liver disease and severe neurological retardation

(2018) J. Gödiker et al.   JOURNAL OF HUMAN GENETICS

Mitochondrial dysfunction in obesity

(2018) Aline Haas de Mello et al.   LIFE SCIENCES

PINK1 Phosphorylates MIC60/Mitofilin to Control Structural Plasticity of Mitochondrial Crista Junctions

(2018) Pei-I Tsai et al.   MOLECULAR CELL

Ablation of the stress protease OMA1 protects against heart failure in mice

(2018) Rebeca Acin-Perez et al.   Science Translational Medicine

Sam50 Regulates PINK1-Parkin-Mediated Mitophagy by Controlling PINK1 Stability and Mitochondrial Morphology

(2018) Fenglei Jian et al.   Cell Reports

Integration of Multi-omics Data from Mouse Diversity Panel Highlights Mitochondrial Dysfunction in Non-alcoholic Fatty Liver Disease

(2018) Karthickeyan Chella Krishnan et al.   Cell Systems

Barth syndrome cells display widespread remodeling of mitochondrial complexes without affecting metabolic flux distribution

(2018) Iliana A. Chatzispyrou et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Mitochondrial inner membrane protein, Mic60/mitofilin in mammalian organ protection

(2018) Yansheng Feng et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Loss of MICOS complex integrity and mitochondrial damage, but not TDP-43 mitochondrial localisation, are likely associated with severity of CHCHD10-related diseases

(2018) Emmanuelle C. Genin et al.   NEUROBIOLOGY OF DISEASE

PD-linked CHCHD2 mutations impair CHCHD10 and MICOS complex leading to mitochondria dysfunction

(2018) Wei Zhou et al.   HUMAN MOLECULAR GENETICS

EGCG protects cardiomyocytes against hypoxia-reperfusion injury via inhibiting OMA1 activation

(2018) Jinliang Nan et al.   JOURNAL OF CELL SCIENCE

Identification and characterization of protein N-myristoylation occurring on four human mitochondrial proteins, SAMM50, TOMM40, MIC19, and MIC25

(2018) Toshihiko Utsumi et al.   PLoS One

Diabetic Cardiomyopathy: Current and Future Therapies. Beyond Glycemic Control

(2018) Giulia Borghetti et al.   Frontiers in Physiology

In vitro and in vivo studies of the ALS-FTLD protein CHCHD10 reveal novel mitochondrial topology and protein interactions

(2017) S R Burstein et al.   HUMAN MOLECULAR GENETICS

Loss of SLC25A46 causes neurodegeneration by affecting mitochondrial dynamics and energy production in mice

(2017) Zhuo Li et al.   HUMAN MOLECULAR GENETICS

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

Mic10, a Core Subunit of the Mitochondrial Contact Site and Cristae Organizing System, Interacts with the Dimeric F 1 F o -ATP Synthase

(2017) Heike Rampelt et al.   JOURNAL OF MOLECULAR BIOLOGY

Rapid degradation of mutant SLC25A46 by the ubiquitin-proteasome system results in MFN1/2-mediated hyperfusion of mitochondria

(2017) Janos Steffen et al.   MOLECULAR BIOLOGY OF THE CELL

Regulated membrane remodeling by Mic60 controls formation of mitochondrial crista junctions

(2017) Manuel Hessenberger et al.   Nature Communications

Loss of Parkinson’s disease-associated protein CHCHD2 affects mitochondrial crista structure and destabilizes cytochrome c

(2017) Hongrui Meng et al.   Nature Communications

Bovine and murine models highlight novel roles for SLC25A46 in mitochondrial dynamics and metabolism, with implications for human and animal health

(2017) Amandine Duchesne et al.   PLoS Genetics

Novel insights into SLC25A46-related pathologies in a genetic mouse model

(2017) Maria Eirini Terzenidou et al.   PLoS Genetics

Changes of mitochondrial ultrastructure and function during ageing in mice and Drosophila

(2017) Tobias Brandt et al.   eLife

Mitochondrial composition and function under the control of hypoxia

(2017) Dominik C. Fuhrmann et al.   Redox Biology

Parkinson disease

(2017) Werner Poewe et al.   Nature Reviews Disease Primers

Adult-onset obesity is triggered by impaired mitochondrial gene expression

(2017) Kara L. Perks et al.   Science Advances

Mitochondria and Cancer

(2016) Sejal Vyas et al.   CELL

The MICOS complex of human mitochondria

(2016) Vera Kozjak-Pavlovic   CELL AND TISSUE RESEARCH

Mitochondrial contact site and cristae organizing system

(2016) Martin van der Laan et al.   CURRENT OPINION IN CELL BIOLOGY

SLC25A46is required for mitochondrial lipid homeostasis and cristae maintenance and is responsible for Leigh syndrome

(2016) Alexandre Janer et al.   EMBO Molecular Medicine

FATE1 antagonizes calcium‐ and drug‐induced apoptosis by uncoupling ER and mitochondria

(2016) Mabrouka Doghman‐Bouguerra et al.   EMBO REPORTS

Mitochondrial hepato-encephalopathy due to deficiency of QIL1/MIC13 (C19orf70), a MICOS complex subunit

(2016) Avraham Zeharia et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Disrupted in schizophrenia 1 (DISC1) is a constituent of the mammalian mitochondrial contact site and cristae organizing system (MICOS) complex, and is essential for oxidative phosphorylation

(2016) Estefanía Piñero-Martos et al.   HUMAN MOLECULAR GENETICS

Loss of OMA1 delays neurodegeneration by preventing stress-induced OPA1 processing in mitochondria

(2016) Anne Korwitz et al.   JOURNAL OF CELL BIOLOGY

CHCHD10 mutations and motor neuron disease: the distribution in Finnish patients

(2016) Sini Penttilä et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Plasticity in mitochondrial cristae density allows metabolic capacity modulation in human skeletal muscle

(2016) Joachim Nielsen et al.   JOURNAL OF PHYSIOLOGY-LONDON

PKA Regulates PINK1 Stability and Parkin Recruitment to Damaged Mitochondria through Phosphorylation of MIC60

(2016) Shiori Akabane et al.   MOLECULAR CELL

Identification of CHCHD10 Mutation in Chinese Patients with Alzheimer Disease

(2016) Tingting Xiao et al.   MOLECULAR NEUROBIOLOGY

Mutation Screening of the CHCHD10 Gene in Chinese Patients with Amyotrophic Lateral Sclerosis

(2016) QingQing Zhou et al.   MOLECULAR NEUROBIOLOGY

Mic60/mitofilin overexpression alters mitochondrial dynamics and attenuates vulnerability of dopaminergic cells to dopamine and rotenone

(2016) Victor S. Van Laar et al.   NEUROBIOLOGY OF DISEASE

Mitochondrial Cristae: Where Beauty Meets Functionality

(2016) Sara Cogliati et al.   TRENDS IN BIOCHEMICAL SCIENCES

Deciphering the Molecular Signals of PINK1/Parkin Mitophagy

(2016) Thanh N. Nguyen et al.   TRENDS IN CELL BIOLOGY

QIL1 mutation causes MICOS disassembly and early onset fatal mitochondrial encephalopathy with liver disease

(2016) Virginia Guarani et al.   eLife

Mitochondrial Dynamics in Diabetic Cardiomyopathy

(2015) Chad A. Galloway et al.   ANTIOXIDANTS & REDOX SIGNALING

The non-glycosylated isoform of MIC26 is a constituent of the mammalian MICOS complex and promotes formation of crista junctions

(2015) Sebastian Koob et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Mic60/Mitofilin determines MICOS assembly essential for mitochondrial dynamics and mtDNA nucleoid organization

(2015) H Li et al.   CELL DEATH AND DIFFERENTIATION

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

Impaired mitochondrial oxidative phosphorylation and supercomplex assembly in rectus abdominis muscle of diabetic obese individuals

(2015) Ghadi Antoun et al.   DIABETOLOGIA

CHCHD10 mutations promote loss of mitochondrial cristae junctions with impaired mitochondrial genome maintenance and inhibition of apoptosis

(2015) E. C. Genin et al.   EMBO Molecular Medicine

Complex-I Alteration and Enhanced Mitochondrial Fusion Are Associated With Prostate Cancer Progression

(2015) Julie V. Philley et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Transgenic overexpression of mitofilin attenuates diabetes mellitus-associated cardiac and mitochondria dysfunction

(2015) Dharendra Thapa et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

CHCHD2 mutations in autosomal dominant late-onset Parkinson's disease: a genome-wide linkage and sequencing study

(2015) Manabu Funayama et al.   LANCET NEUROLOGY

Targeting mitochondria metabolism for cancer therapy

(2015) Samuel E Weinberg et al.   Nature Chemical Biology

Mutations in SLC25A46, encoding a UGO1-like protein, cause an optic atrophy spectrum disorder

(2015) Alexander J Abrams et al.   NATURE GENETICS

The Roles of PINK1, Parkin, and Mitochondrial Fidelity in Parkinson’s Disease

(2015) Alicia M. Pickrell et al.   NEURON

Detailed Analysis of the Human Mitochondrial Contact Site Complex Indicate a Hierarchy of Subunits

(2015) Christine Ott et al.   PLoS One

Mitochondrial biogenesis is required for the anchorage-independent survival and propagation of stem-like cancer cells

(2015) Arianna De Luca et al.   Oncotarget

Mitofilin and CHCHD6 physically interact with Sam50 to sustain cristae structure

(2015) Chengli Ding et al.   Scientific Reports

Suppression of Mic60 compromises mitochondrial transcription and oxidative phosphorylation

(2015) Rui-Feng Yang et al.   Scientific Reports

MICOS coordinates with respiratory complexes and lipids to establish mitochondrial inner membrane architecture

(2015) Jonathan R Friedman et al.   eLife

QIL1 is a novel mitochondrial protein required for MICOS complex stability and cristae morphology

(2015) Virginia Guarani et al.   eLife

Late onset spinal motor neuronopathy is caused by mutation inCHCHD10

(2014) Sini Penttilä et al.   ANNALS OF NEUROLOGY

A mitochondrial origin for frontotemporal dementia and amyotrophic lateral sclerosis through CHCHD10 involvement

(2014) Sylvie Bannwarth et al.   BRAIN

Silencing of mitochondrial Lon protease deeply impairs mitochondrial proteome and function in colon cancer cells

(2014) Lara Gibellini et al.   FASEB JOURNAL

Uniform nomenclature for the mitochondrial contact site and cristae organizing system

(2014) Nikolaus Pfanner et al.   JOURNAL OF CELL BIOLOGY

Apolipoprotein O is mitochondrial and promotes lipotoxicity in heart

(2014) Annie Turkieh et al.   JOURNAL OF CLINICAL INVESTIGATION

A Splicing Mutation in the Novel Mitochondrial Protein DNAJC11 Causes Motor Neuron Pathology Associated with Cristae Disorganization, and Lymphoid Abnormalities in Mice

(2014) Fotis Ioakeimidis et al.   PLoS One

Mitochondrial Dynamics in the Regulation of Nutrient Utilization and Energy Expenditure

(2013) Marc Liesa et al.   Cell Metabolism

Mitochondrial adaptations and dysfunctions in nonalcoholic fatty liver disease

(2013) Karima Begriche et al.   HEPATOLOGY

Dysfunctional cardiac mitochondrial bioenergetic, lipidomic, and signaling in a murine model of Barth syndrome

(2013) Michael A. Kiebish et al.   JOURNAL OF LIPID RESEARCH

Quantitative Proteomic and Functional Analysis of Liver Mitochondria from High Fat Diet (HFD) Diabetic Mice

(2013) Yurong Guo et al.   MOLECULAR & CELLULAR PROTEOMICS

Diagnosis of mitochondrial myopathies

(2013) Margherita Milone et al.   MOLECULAR GENETICS AND METABOLISM

NAFLD, NASH and liver cancer

(2013) Gregory A. Michelotti et al.   Nature Reviews Gastroenterology & Hepatology

Mitochondrial protein ATPase family, AAA domain containing 3A correlates with radioresistance in glioblastoma

(2013) W.-C. You et al.   NEURO-ONCOLOGY

Barth syndrome

(2013) Sarah LN Clarke et al.   Orphanet Journal of Rare Diseases

APOOL Is a Cardiolipin-Binding Constituent of the Mitofilin/MINOS Protein Complex Determining Cristae Morphology in Mammalian Mitochondria

(2013) Tobias A. Weber et al.   PLoS One

Motor Control Abnormalities in Parkinson's Disease

(2013) P. Mazzoni et al.   Cold Spring Harbor Perspectives in Medicine

Mitofilin regulates cytochrome c release during apoptosis by controlling mitochondrial cristae remodeling

(2012) Rui-feng Yang et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

SnapShot: Mitochondrial Architecture

(2012) Walter Neupert   CELL

CHCM1/CHCHD6, Novel Mitochondrial Protein Linked to Regulation of Mitofilin and Mitochondrial Cristae Morphology

(2012) Jie An et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Restricted diffusion of OXPHOS complexes in dynamic mitochondria delays their exchange between cristae and engenders a transitory mosaic distribution

(2012) Verena Wilkens et al.   JOURNAL OF CELL SCIENCE

Sam50 Functions in Mitochondrial Intermembrane Space Bridging and Biogenesis of Respiratory Complexes

(2012) C. Ott et al.   MOLECULAR AND CELLULAR BIOLOGY

Alterations in Skeletal Muscle Indicators of Mitochondrial Structure and Biogenesis in Patients with Type 2 Diabetes and Heart Failure: Effects of Epicatechin Rich Cocoa

(2011) Pam R. Taub et al.   CTS-Clinical and Translational Science

Dual Role of Mitofilin in Mitochondrial Membrane Organization and Protein Biogenesis

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

The mitochondrial contact site complex, a determinant of mitochondrial architecture

(2011) Max Harner et al.   EMBO JOURNAL

A mitochondrial-focused genetic interaction map reveals a scaffold-like complex required for inner membrane organization in mitochondria

(2011) Suzanne Hoppins et al.   JOURNAL OF CELL BIOLOGY

Effects of high-fat diet and regular aerobic exercise on global gene expression in skeletal muscle of C57BL/6 mice

(2011) Li Fu et al.   METABOLISM-CLINICAL AND EXPERIMENTAL

Proteomic alterations of distinct mitochondrial subpopulations in the type 1 diabetic heart: contribution of protein import dysfunction

(2010) Walter A. Baseler et al.   AMERICAN JOURNAL OF PHYSIOLOGY-REGULATORY INTEGRATIVE AND COMPARATIVE PHYSIOLOGY

ChChd3, an Inner Mitochondrial Membrane Protein, Is Essential for Maintaining Crista Integrity and Mitochondrial Function

(2010) Manjula Darshi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cardiac and Skeletal Muscle Defects in a Mouse Model of Human Barth Syndrome

(2010) Devrim Acehan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

ATPase family AAA domain-containing 3A is a novel anti-apoptotic factor in lung adenocarcinoma cells

(2010) H.-Y. Fang et al.   JOURNAL OF CELL SCIENCE

Disrupted-in-schizophrenia1 (DISC1) plays essential roles in mitochondria in collaboration with Mitofilin

(2010) Young-Un Park et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Deficiency of electron transport chain in human skeletal muscle mitochondria in type 2 diabetes mellitus and obesity

(2009) Vladimir B. Ritov et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM

Gene Expression Profile Prospectively Predicts Peritoneal Relapse After Curative Surgery of Gastric Cancer

(2009) Atsushi Takeno et al.   ANNALS OF SURGICAL ONCOLOGY

Proteomics Analysis of Human Skeletal Muscle Reveals Novel Abnormalities in Obesity and Type 2 Diabetes

(2009) H. Hwang et al.   DIABETES

Regulation of OPA1 processing and mitochondrial fusion bym-AAA protease isoenzymes and OMA1

(2009) Sarah Ehses et al.   JOURNAL OF CELL BIOLOGY

Inducible proteolytic inactivation of OPA1 mediated by the OMA1 protease in mammalian cells

(2009) Brian Head et al.   JOURNAL OF CELL BIOLOGY

Formation of cristae and crista junctions in mitochondria depends on antagonism between Fcj1 and Sue/g

(2009) Regina Rabl et al.   JOURNAL OF CELL BIOLOGY

Dimer ribbons of ATP synthase shape the inner mitochondrial membrane

(2008) Mike Strauss et al.   EMBO JOURNAL

Nonalcoholic fatty liver disease and mitochondrial dysfunction

(2008) Yongzhong Wei et al.   WORLD JOURNAL OF GASTROENTEROLOGY