Lanz et al. used mass spectrometry to measure how proteome composition changes with cell size (i.e., DNA-to-volume ratio). They found that different cellular components scale differently with size....

Nature Reviews Genetics - Rackham and Filipovska review our current understanding of mitochondrial genome organization and expression, and discuss how new technologies and animal models are...

Dedicated protein quality control systems ensure the folding of newly synthesized proteins at ribosomes. Kohler et al. reveal such a system for mitochondrially encoded proteins in the form of the...

Nature - The mechanisms by which mitochondria are transferred between cells and how intercellular mitochondria transfer regulates physiological processes and disease pathogenesis are discussed.

Song et al. presented an optogenetic method which utilizes light-mediated recruitment of molecular motors to induce mitochondria deformation in live cells with high spatial resolution and general...

Nature Communications - How age-accumulated mtDNA mutations in the small intestine modulate intestinal homeostasis is unclear. Here, the authors show that increased mtDNA mutation burden triggers...

image image Mitochondrial respiratory supercomplexes are found in various organisms, however their physiological function remains elusive. This study reveals that supercomplexes enhance mitochondrial...

Cellular homeostasis requires the sensing of and adaptation to intracellular oxygen (O2) and reactive oxygen species (ROS). The Arg/N-degron pathwa...

Mitochondria form a complex, interconnected reticulum that is maintained through coordination among biogenesis, dynamic fission, and fusion and mit...

Nature Communications - Aerobic energy metabolism is driven by proton-pumping respiratory supercomplexes. The study reports the structural basis for energy conversion in such supercomplex. It may...

Nature Reviews Genetics - The authors review the field of mammalian mitochondrial genome engineering, culminating in the recent development of mitochondrially targeted programmable nucleases and...

Nature Communications - Mutations in mitochondrial DNA can lead to clinically heterogeneous disease. Here the authors demonstrate in vivo base editing of mouse mitochondrial DNA in a post-mitotic...

Nature Aging - This Review provides an update on the pleiotropic effects of mitochondria in aging and discusses how defects in mitochondrial stress pathways contribute to the decline in cellular...

Cell Death & Disease - Dynamic regulation of mitochondrial-endoplasmic reticulum crosstalk during stem cell homeostasis and aging

Nature Communications - It has been a longstanding goal to promote the propagation of functional mitochondrial DNAs at the expense of pathological molecules in cells where the two species coexist....

Nature Communications - The mitochondrial ADP/ATP carrier transports adenine nucleotides. Here, authors identify the residues involved in substrate binding. One set forms the central substrate...

Nature Communications - Membrane contact sites between organelles have specialized functions that are only beginning to be understood. Here, the authors show that ORP1L mediates lysosome...

Aging is a process of progressive change. In order to develop biological models of aging, longitudinal datasets with high temporal resolution are needed. Here we report a multi-omic longitudinal...

Nature Chemistry - Elucidating the origin of translation—the process that produces a specific peptide from an RNA sequence—is one of the most difficult challenges in prebiotic chemistry...

Nature - SCAF1 is solely required for supercomplex CIII2CIV assembly and is not involved in the formation of the respirasome (supercomplex CICIII2CIV)

Nature Structural & Molecular Biology - The folding of ribosomal RNAs is central to the biogenesis of the mitoribosome and is a complex, stepwise process. Five recent cryo-EM studies detail the...

STAR Protocols is an open access, peer-reviewed journal from Cell Press. We offer structured, transparent, accessible, and repeatable step-by-step experimental and computational protocols from all...

Nature - Dysfunction of mitochondrial complex I in mice is sufficient to cause progressive parkinsonism in which the loss of nigral dopamine release critically contributes to motor dysfunction.

Cell Death & Disease - Membrane-tethering of cytochrome c accelerates regulated cell death in yeast

Fumarate is a terminal electron acceptor in the mammalian electron transport chain, and its reduction sustains mitochondrial functions in hypoxia.

Brüser et al. report that the numerous nucleoids (mitochondrial DNA-protein complexes) located in the mitochondrial network of a single cell differ with respect to their replication and transcription...

Homologous-recombination-deficient (HRD) tumors often develop resistance to traditional chemotherapies, making them difficult to kill. Kovacs et al. find that HRD tumor cells exhibit DNA-damage-ind...

Krishna et al. discover that mitochondria in neurons and muscle fibers exhibit slow turnover and contain many long-lived proteins that can be part of large protein complexes. The longevity of these...

Lee et al. show that autophagy proteins regulate lysosome size and function in response to metabolic stress by controlling selective turnover of the lysosomal membrane. Selective turnover occurs...

Nature Communications - The most common protein modification in eukaryotes is N-terminal acetylation, but its functional impact has remained enigmatic. Here, the authors find that a key role for...

image image This study presents a non‐radioactive mitochondrial import assay for matrix proteins using import substrates conjugated to a fluorophore. The method is a fast and safe approach to monitor...

Nature Communications - Here the authors describe high depth mitochondrial DNA (mtDNA) sequence analysis of 146 human induced pluripotent stem cell (hiPSC) lines as well as single cell RNA-seq...

Nature Communications - During aging, proteins are damaged and can misfold, compromising cellular viability. Here, Kohler et al. uncover how aging cells maintain fitness by redirecting the protein...

Nature Reviews Molecular Cell Biology - Mitochondrial respiration relies on five enzymatic complexes that couple electron transport with proton pumping, leading to ATP synthesis. Recent studies...

Mitochondria are crucial for cellular energy metabolism and human health. Morgenstern et al. present a high-confidence protein compendium of human mitochondria including mitochondria-specific protein...

The mitochondrial respiratory chain is composed of nuclear as well as mitochondrial-encoded subunits. A variety of factors mediate co-translational integration of mtDNA-encoded proteins into the...

Nature - During senescence, minority mitochondrial outer membrane permeabilization leads to the release of mtDNA into the cytosol through BAX and BAK macropores, in turn activating the...

Nature Structural & Molecular Biology - Fluorescence imaging and fluorescence recovery after photobleaching assays reveal that the mitochondrial nucleoid forms a suborganelle via...

Nature Medicine - The association between certain mitochondrial DNA variants and increased risk of late-onset diseases in humans could be explained by a direct role of mitochondrial DNA in the...

Background The establishment and maintenance of functional neural connections relies on appropriate distribution and localization of mitochondria in neurites, as these organelles provide essential...

The Kohler Mito Lab, led by Andreas Kohler at the Department of Medical Biochemistry and Biophysics, Umeå University, Sweden. We investigate molecular principles of mitochondrial protein quality...

Mitochondrial protein import in the parasitic protozoan Trypanosoma brucei is mediated by the atypical outer membrane translocase, ATOM. It consists of seven subunits including ATOM69, the import...

The mitochondria contain their own genome derived from an alphaproteobacterial endosymbiont. From thousands of protein-coding genes originally encoded by their ancestor, only between 1 and about 70...

Cytochrome c oxidase assembly factor 7 (COA7) is a metazoan-specific assembly factor, critical for the biogenesis of mitochondrial complex IV (cytochrome c oxidase). Although mutations in COA7 have...

Nature - We quantify mitochondrial DNA copy number and heteroplasmy levels and study their association with nuclear genetic loci in population-scale biobanks.

image image A CRISPR‐Cas9 screen shows that mTORC1 loss increases OXPHOS and prevents IMT1‐induced cell death whereas loss of VHL confers resistance in an OXPHOS‐independent fashion. A CRISPR‐Cas9...

Nature - Ageing alters the kinetics of translation elongation in both Caenorhabditis elegans and Saccharomyces cerevisiae.

image image AAA proteases regulate key processes in mitochondria and allow dynamic reshaping of the mitochondrial proteome. This study reports identification of TMBIM5 (GHITM, MICS1) as a Ca2+/H+...

Mitochondrial carrier homolog 2 mediates the insertion of diverse alpha-helical proteins into the mitochondrial outer membrane.

AbstractMotivation. Cells respond to environments by regulating gene expression to exploit resources optimally. Recent advances in technologies allow for m

Mitochondrial protein biosynthesis is maintained by an interplay between the mitochondrial ribosome (mitoribosome) and a large set of protein interaction partners. This interactome regulates a...