An evolutionarily conserved mitochondrial haem-sensing mechanism that predates the emergence of haemoglobin-based oxygen transport could have therapeutic value in iron deficiency disorders.

Mitochondrial function depends on the maintenance of its genome, and disruptions in copy number and distribution are hallmarks of mitochondrial disorders. Mitochondrial DNA (mtDNA) replication is spat...

Mitochondrial disease can result from mutations in the enzymes responsible for biosynthesis of heme a and hemylation of respiratory complex IV of the electron transport chain, also known as cytochrome...

l-2-Hydroxyglutarate is identified as a legitimate physiological signalling metabolite, and control of its levels is essential for postnatal growth and survival and correct renal development and funct...

Heme, an iron-containing cofactor, is synthesized in mitochondria by an eight-enzyme pathway. Although cells were thought to manage heme autonomously, more than 1000 proteins contribute to its product...

Kroczek et al show that degradation of DNAJC15 by OMA1 and AFG3L2 under stress limits mitochondrial protein import and OXPHOS biogenesis. Non-imported proteins lead to the induction of the unfolded pr...

Extensive metabolite exchanges occur between mitochondria and the cytosol through the action of transporter proteins, which are placed onto the inner mitochondrial membrane by the TIM22 carrier transl...

A forward genetic screen in Caenorhabditis elegans reveals that decreased levels of the ferredoxin FDX2 suppress the loss of frataxin in worms and in mice by relief of FDX2 inhibition of fra...

Cells can sense mitochondrial damage through signalling via an unimported mitochondrial targeting sequence.

Cryogenic electron microscopy structures and functional analyses reveal that NCLX functions as a H+/Ca2+ rather than a Na+/Ca2+ exchanger, and uncover its transport mechanism with implications for the...

Wakigawa et al. present advanced derivatives of mitochondrial ribosome profiling. By quantifying initiation, elongation, and ribosome collisions and by dissecting the impacts of tRNA anticodon-loop mo...

mitoRQC protects mitochondria against toxic CAT-tailed proteins. Using a genome-wide screen, Bertram et al. identify Pth2 as a peptidyl-tRNA hydrolase within mitoRQC. Pth2 modulates the import of CAT-...

Nguyen, Collier et al. find a mitochondria–lysosome inflammatory pathway regulated by the SUMO E3 ligase MAPL, which promotes vesicular mtDNA transport to lysosomes and subsequent gasdermin-dependent ...

Researchers are testing multiple treatments for the rare genetic conditions

Yang et al. uncover the molecular details of how mitochondrial proteins cross the outer and inner mitochondrial membranes, with hydrophobicity guiding them into distinct pathways toward specific desti...

Localized translation broadly enables spatiotemporal control of gene expression. Here, we present LOV-domain-controlled ligase for translation localiz…

Research on Drosophila neurons shows links between the need to sleep and aerobic metabolism, indicating that the pressure to sleep may have a mitochondrial origin.

AIFM1 is crucial for mitochondrial function. Here, Rothemann, Pavlenko et al. show that AK2A interacts with NADH-dependent AIFM1 dimers. They demonstrate that this interaction stabilizes AIFM1 dimers ...

Rashan, Bartlett and colleagues show that mammalian 4-hydroxy fatty acids are primarily catabolized by ACAD10 and ACAD11 (atypical mitochondrial and peroxisomal acyl-CoA dehydrogenases, respectively) ...

Human OPA1 embeds itself into cardiolipin-containing membranes through a lipid-binding paddle domain, and OPA1 oligomerization through multiple assembly interfaces promotes the helical assembly of a f...

imageimageBcs1 is an unusual, heptameric AAA-ATPase that translocates its cargo, Rip1, across the inner mitochondrial membrane in a fully folded state. Cryo-EM and functional data provide novel insigh...

Mitochondria fulfill central functions in metabolism and energy supply. They express their own genome, which encodes key subunits of the oxidative phosphorylation system. However, central mechanisms u...

The inner mitochondrial membrane forms cristae, which are crucial for mitochondrial function. This Review explores the protein complexes that regulate cristae dynamics, including remodelling and fusio...

Stomatin, Prohibitin, Flotillin, and HflK/C (SPFH) family proteins are found in all kingdoms of life and in multiple eukaryotic organelles. SPFH proteins assemble into homo- or hetero-oligomeric rings...

The mitochondrial pyruvate carrier transport mechanism is ΔpH driven and is inhibited competitively by distinct compound classes.

Horses are among nature’s greatest athletes, yet the ancestral molecular adaptations fueling their energy demands are poorly understood. Within a clinically important pathway regulating redox and meta...

Systematic base-editing and computational screens identify specific cysteine residues on VPS35 in the retromer complex as key sensors that decrease mitochondrial translation in response to reactive ox...

Proceedings of the National Academy of Sciences (PNAS), a peer reviewed journal of the National Academy of Sciences (NAS) - an authoritative source of high-impact, original research that broadly spans...

Lange, Ratz, et al. investigate the number and distribution of human prohibitin complexes in the mitochondrial inner membrane, uncovering their bell-shaped structure and assembly of alternating PHB1 a...

Mitochondria regenerate adenosine triphosphate (ATP) through oxidative phosphorylation. This process is carried out by five membrane-bound complexes collectively known as the respiratory chain, workin...

Mutations in the ubiquitin kinase PINK1 cause early onset Parkinson’s Disease, but how PINK1 is stabilized at depolarized mitochondrial translocase complexes has remained poorly understood. We determi...

Peroxiredoxins are highly conserved thiol peroxidases essential for peroxide detoxification, redox signaling, and chaperone activity. Prx1/AhpC-type peroxiredoxins are found throughout the eukaryotic ...

Combined expression of a mitochondrially targeted endonuclease and end-joining machinery enables formation of mtDNA deletions with a broad range of heteroplasmy.

Structural findings define the architecture of the mitochondrial pyruvate carrier, delineate its substrate-binding site and translocation pathway, and reveal its major conformational states, providing...

Rigoni et al. use complexomic to develop two online-available tools, MARIGOLD and MitoCIAO, mapping mitochondrial protein interactions during membrane remodeling. MARIGOLD provides “digital western bl...

American Society for Biochemistry and Molecular Biology honors 24 members for their service to the society and accomplishments in research, education, mentorship, diversity and inclusion and advocacy.

Marlar-Pavey et al. determine that a subpopulation of compositionally unique mitochondria, METEORs, dynamically localize to filopodia. Loss of mitochondrial targeting to filopodia correlates with a re...

We find that, in mice, although the individual loss of Parkin or OMA1 does not affect mitochondrial integrity, their combined loss results in small body size, low locomotor activity, premature death, ...

A small molecule that increases oxygen-hemoglobin affinity was shown to prevent and reverse pathology in a mouse model of mitochondrial disease, thereby enabling a practical form of hypoxia therapy.

Mitochondrial DNA (mtDNA) copy number regulation remains incompletely understood, despite its importance in cellular function. In Saccharomyces cerevisiae, Mrx6 belongs to the Pet20-domain-containing ...

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