Khalimonchuk Lab

Stress adaptation of mitochondrial protein import by OMA1-mediated degradation of DNAJC15 - Nature Structural & Molecular Biology 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...

Stress adaptation of mitochondrial protein import by OMA1-mediated degradation of DNAJC15

Structural evolution of the MTCH family of mitochondrial insertases.

The TIM22 carrier translocase supports cell proliferation by facilitating mitochondrial iron uptake for Fe-S biogenesis 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...

The TIM22 carrier translocase supports cell proliferation by facilitating mitochondrial iron uptake for Fe-S biogenesis

Mutations in mitochondrial ferredoxin FDX2 suppress frataxin deficiency - Nature 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...

Mutations in mitochondrial ferredoxin FDX2 suppress frataxin deficiency

A direct role for a mitochondrial targeting sequence in signalling stress - Nature Cells can sense mitochondrial damage through signalling via an unimported mitochondrial targeting sequence.

A direct role for a mitochondrial targeting sequence in signalling stress

Structure and mechanism of the mitochondrial calcium transporter NCLX - Nature 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...

Structure and mechanism of the mitochondrial calcium transporter NCLX.

Monitoring the complexity and dynamics of mitochondrial translation 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...

Monitoring the complexity and dynamics of mitochondrial translation

Delayed protein translocation protects mitochondria against toxic CAT-tailed proteins 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-...

Delayed protein translocation protects mitochondria against toxic CAT-tailed proteins

MAPL regulates gasdermin-mediated release of mtDNA from lysosomes to drive pyroptotic cell death - Nature Cell Biology 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 ...

MAPL regulates gasdermin-mediated release of mtDNA from lysosomes to drive pyroptotic cell death

First approved drug for mitochondrial disease could pave way for more treatments Researchers are testing multiple treatments for the rare genetic conditions

Mitochondrial diseases have seemed intractable, but last month the Food and Drug Administration OK’d the first treatment targeting a mitochondrial flaw. https://scim.ag/4q3Ymxz

Dynamic TOM–TIM23 supercomplex directs mitochondrial protein translocation and sorting - Nature Structural & Molecular Biology 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...

Dynamic TOM–TIM23 supercomplex directs mitochondrial protein translocation and sorting.

Proximity-specific ribosome profiling reveals the logic of localized mitochondrial translation Localized translation broadly enables spatiotemporal control of gene expression. Here, we present LOV-domain-controlled ligase for translation localiz…

Proximity-specific ribosome profiling reveals the logic of localized mitochondrial translation

Super-resolution microscopy of mitochondrial mRNAs

Mitochondrial origins of the pressure to sleep - Nature 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.

Mitochondrial origins of the pressure to sleep

Congrats, Judi!!

Small molecule OPA1 inhibitors amplify cytochrome c release and reverse cancer cells resistance to Bcl-2 inhibitors

Interaction with AK2A links AIFM1 to cellular energy metabolism 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 ...

Interaction with AK2A links AIFM1 to cellular energy metabolism

ACAD10 and ACAD11 enable mammalian 4-hydroxy acid lipid catabolism - Nature Structural & Molecular Biology 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) ...

ACAD10 and ACAD11 enable mammalian 4-hydroxy acid lipid catabolism

Structural mechanism of mitochondrial membrane remodelling by human OPA1 - Nature 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...

🔥🔥 Structural mechanism of mitochondrial membrane remodelling by human OPA1

Excited to visit LMU!

Mechanistic insights into Bcs1-mediated mitochondrial membrane translocation of the folded Rieske protein | The EMBO Journal 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...

Mechanistic insights into Bcs1-mediated mitochondrial membrane translocation of the folded Rieske protein

Silencing mitochondrial gene expression in living cells 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...

Researchers in Science have developed a tool to silence specific mitochondria-encoded mRNAs in living cells, which could help scientists understand the origins of certain mitochondria-related diseases. scim.ag/3F6Wm5d

What complex are you if you have a very versatile company in close proximity? The MICOS Complex.
We present the MINDNet: Proximity Proteomics revealing the nanodomain interactome in the mitochondrial inner membrane, done by the talented Yulia and team.

Molecular machineries shaping the mitochondrial inner membrane - Nature Reviews Molecular Cell Biology 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...

Molecular machineries shaping the mitochondrial inner membrane.

Fmp30 is a phosphatidylinositol hydrolase that regulates CoQ biosynthesis https://www.biorxiv.org/content/10.1101/2025.05.01.651778v1

Structures of human organellar SPFH protein complexes 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...

Structures of human organellar SPFH protein complexes

Molecular basis of pyruvate transport and inhibition of the human mitochondrial pyruvate carrier The mitochondrial pyruvate carrier transport mechanism is ΔpH driven and is inhibited competitively by distinct compound classes.

Molecular basis of pyruvate transport and inhibition of the human mitochondrial pyruvate carrier

Small molecules restore mutant mitochondrial DNA polymerase activity

Running a genetic stop sign accelerates oxygen metabolism and energy production in horses 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...

Running a genetic stop sign accelerates oxygen metabolism and energy production in horses

Oxidation of retromer complex controls mitochondrial translation - Nature 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...

Oxidation of retromer complex controls mitochondrial translation