Khalimonchuk Lab
@mitocrue.bsky.social
Semper vestigans | All things mitochondria https://biochem.unl.edu/oleh-khalimonchuk-lab/
Really hyped to share our pre-print on mtDNA replication-fission ! @nanigrotjahn.bsky.social (doi: doi.org/10.64898/202...) 🧵with details below ⤵️
ATAD3A structurally links mtDNA replication and mitochondrial fission
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...
doi.org
Our latest publication is out. Kudos to all involved!
Molecular analysis of a new patient COX15 mutation provides insight into the etiology of fatal infantile cardioencephalopathy
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...
jbc.org
Mitochondrial L-2-hydroxyglutarate is a physiological signalling metabolite
Mitochondrial l-2-hydroxyglutarate is a physiological signalling metabolite - Nature
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...
nature.com
A cell-nonautonomous heme acquisition pathway enables erythroid hemoglobinization under stress.
A cell-nonautonomous heme acquisition pathway enables erythroid hemoglobinization under stress
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...
science.org
🔬 You helped move the field forward at ASBMB 2026. 💬 Ready to keep the conversation going? Submit your idea for a mini-symposium, interest group session or workshop for ASBMB 2027. ➡️ www.asbmb.org/annual-meeti... #ASBMB27 #ResearchCommunity #ScientificConference
Proposals
asbmb.org
Stress adaptation of mitochondrial protein import by OMA1-mediated degradation of DNAJC15
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...
nature.com
The TIM22 carrier translocase supports cell proliferation by facilitating mitochondrial iron uptake for Fe-S biogenesis
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...
cell.com
Mutations in mitochondrial ferredoxin FDX2 suppress frataxin deficiency
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...
nature.com
A direct role for a mitochondrial targeting sequence in signalling stress
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.
nature.com
Structure and mechanism of the mitochondrial calcium transporter NCLX.
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...
nature.com
Monitoring the complexity and dynamics of mitochondrial translation
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...
cell.com
Delayed protein translocation protects mitochondria against toxic CAT-tailed proteins
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-...
cell.com
MAPL regulates gasdermin-mediated release of mtDNA from lysosomes to drive pyroptotic cell death
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 ...
nature.com
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
First approved drug for mitochondrial disease could pave way for more treatments
Researchers are testing multiple treatments for the rare genetic conditions
scim.ag
Dynamic TOM–TIM23 supercomplex directs mitochondrial protein translocation and sorting.
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...
nature.com
Proximity-specific ribosome profiling reveals the logic of localized mitochondrial translation
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…
sciencedirect.com
Small molecule OPA1 inhibitors amplify cytochrome c release and reverse cancer cells resistance to Bcl-2 inhibitors
https://www.science.org/doi/10.1126/sciadv.adx4562?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed
t.co
Interaction with AK2A links AIFM1 to cellular energy metabolism
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 ...
cell.com
ACAD10 and ACAD11 enable mammalian 4-hydroxy acid lipid catabolism
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) ...
nature.com
🔥🔥 Structural mechanism of mitochondrial membrane remodelling by human OPA1
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...
nature.com
Excited to visit LMU!
Gene Center Munich @LMU Munich@genecenter-lmu.bsky.social · last yr.📣Our guest series continues with Oleh Khalimonchuk! We're happy to welcome him next Monday! 🎉 He’ll be talking about “Regulation of mitochondrial shape and function: From basics to disease” 📅 June 2 🕐 1:15 PM ➡️Lynen Lecture Hall Don’t miss it! 🤗✨ @mitocrue.bsky.social @jaelab.bsky.social
Mechanistic insights into Bcs1-mediated mitochondrial membrane translocation of the folded Rieske protein
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...
embopress.org
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
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...
scim.ag
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.
bioRxiv Cell Biology@biorxiv-cellbio.bsky.social · last yr.MINDNet: Proximity interactome of the MICOS complex revealing a multifaceted network orchestrating mitochondrial biogenesis https://www.biorxiv.org/content/10.1101/2025.05.20.655052v1
Molecular machineries shaping the mitochondrial inner membrane.
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...
nature.com
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
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...
biorxiv.org
