Khalimonchuk Lab
@mitocrue.bsky.social
📤 987
📥 505
📝 55
Semper vestigans | All things mitochondria
https://biochem.unl.edu/oleh-khalimonchuk-lab/
Monitoring the complexity and dynamics of mitochondrial translation
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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...
https://www.cell.com/molecular-cell/abstract/S1097-2765(25)00863-9?rss=yes
16 days ago
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Delayed protein translocation protects mitochondria against toxic CAT-tailed proteins
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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-...
https://www.cell.com/molecular-cell/fulltext/S1097-2765(25)00815-9?rss=yes
about 1 month ago
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MAPL regulates gasdermin-mediated release of mtDNA from lysosomes to drive pyroptotic cell death
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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 ...
https://www.nature.com/articles/s41556-025-01774-y
about 2 months ago
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1
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reposted by
Khalimonchuk Lab
Science Magazine
about 2 months ago
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
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First approved drug for mitochondrial disease could pave way for more treatments
Researchers are testing multiple treatments for the rare genetic conditions
https://scim.ag/4q3Ymxz
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Dynamic TOM–TIM23 supercomplex directs mitochondrial protein translocation and sorting.
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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...
https://www.nature.com/articles/s41594-025-01662-x
3 months ago
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Proximity-specific ribosome profiling reveals the logic of localized mitochondrial translation
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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…
https://www.sciencedirect.com/science/article/pii/S009286742500916X
3 months ago
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Super-resolution microscopy of mitochondrial mRNAs
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https://idp.nature.com/authorize?response_type=cookie
4 months ago
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Mitochondrial origins of the pressure to sleep
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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.
https://www.nature.com/articles/s41586-025-09261-y
5 months ago
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Small molecule OPA1 inhibitors amplify cytochrome c release and reverse cancer cells resistance to Bcl-2 inhibitors
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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
https://t.co/WJdTiajt7p
5 months ago
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Interaction with AK2A links AIFM1 to cellular energy metabolism
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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 ...
https://www.cell.com/molecular-cell/fulltext/S1097-2765(25)00500-3?rss=yes
5 months ago
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ACAD10 and ACAD11 enable mammalian 4-hydroxy acid lipid catabolism
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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) ...
https://www.nature.com/articles/s41594-025-01596-4
5 months ago
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🔥🔥 Structural mechanism of mitochondrial membrane remodelling by human OPA1
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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...
https://www.nature.com/articles/s41586-023-06441-6
6 months ago
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Excited to visit LMU!
add a skeleton here at some point
6 months ago
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Mechanistic insights into Bcs1-mediated mitochondrial membrane translocation of the folded Rieske protein
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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...
https://www.embopress.org/doi/full/10.1038/s44318-025-00459-4
6 months ago
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reposted by
Khalimonchuk Lab
Science Magazine
6 months ago
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
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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...
https://scim.ag/3F6Wm5d
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reposted by
Khalimonchuk Lab
Kondadi Lab
6 months ago
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.
add a skeleton here at some point
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Molecular machineries shaping the mitochondrial inner membrane.
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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...
https://www.nature.com/articles/s41580-025-00854-z
7 months ago
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reposted by
Khalimonchuk Lab
bioRxivpreprint
7 months ago
Fmp30 is a phosphatidylinositol hydrolase that regulates CoQ biosynthesis
https://www.biorxiv.org/content/10.1101/2025.05.01.651778v1
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Structures of human organellar SPFH protein complexes
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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...
https://www.biorxiv.org/content/10.1101/2025.04.21.649849v1
7 months ago
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Molecular basis of pyruvate transport and inhibition of the human mitochondrial pyruvate carrier
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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.
https://www.science.org/doi/10.1126/sciadv.adw1489
8 months ago
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8
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Small molecules restore mutant mitochondrial DNA polymerase activity
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https://www.nature.com/articles/s41586-025-08856-9
https://t.co/dN8YmvxHom
8 months ago
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Running a genetic stop sign accelerates oxygen metabolism and energy production in horses
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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...
https://www.science.org/doi/10.1126/science.adr8589
8 months ago
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Oxidation of retromer complex controls mitochondrial translation
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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...
https://www.nature.com/articles/s41586-025-08756-y
8 months ago
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A splendid molecular factory: De- and reconstruction of the mammalian respiratory chain
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PNAS
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...
https://www.pnas.org/doi/10.1073/pnas.2416162122
8 months ago
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In situ architecture of the human prohibitin complex
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In situ architecture of the human prohibitin complex - Nature Cell Biology
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...
https://www.nature.com/articles/s41556-025-01620-1
8 months ago
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In-cell architecture of the mitochondrial respiratory chain
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In-cell architecture of the mitochondrial respiratory chain
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...
https://www.science.org/doi/10.1126/science.ads8738
8 months ago
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🔥🔥 Structure of human PINK1 at a mitochondrial TOM-VDAC array
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Structure of human PINK1 at a mitochondrial TOM-VDAC array
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...
https://www.science.org/doi/10.1126/science.adu6445
9 months ago
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Heterooligomerization drives structural plasticity of eukaryotic peroxiredoxins
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Heterooligomerization drives structural plasticity of eukaryotic peroxiredoxins
Peroxiredoxins are highly conserved thiol peroxidases essential for peroxide detoxification, redox signaling, and chaperone activity. Prx1/AhpC-type peroxiredoxins are found throughout the eukaryotic ...
https://www.biorxiv.org/content/10.1101/2025.03.03.641172v1
9 months ago
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Engineering mtDNA deletions by reconstituting end joining in human mitochondria
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Engineering mtDNA deletions by reconstituting end joining in human mitochondria
Combined expression of a mitochondrially targeted endonuclease and end-joining machinery enables formation of mtDNA deletions with a broad range of heteroplasmy.
https://www.cell.com/cell/fulltext/S0092-8674%2825%2900194-1
9 months ago
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Structure of mitochondrial pyruvate carrier and its inhibition mechanism
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Structure of mitochondrial pyruvate carrier and its inhibition mechanism - Nature
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...
https://www.nature.com/articles/s41586-025-08667-y
9 months ago
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reposted by
Khalimonchuk Lab
Christian Frezza
9 months ago
MARIGOLD and MitoCIAO, two searchable compendia to visualize and functionalize protein complexes during mitochondrial remodeling: Cell Metabolism
www.cell.com/cell-metabol...
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MARIGOLD and MitoCIAO, two searchable compendia to visualize and functionalize protein complexes during mitochondrial remodeling
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...
https://www.cell.com/cell-metabolism/fulltext/S1550-4131%2825%2900017-8
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My study section meeting just got postponed, which means ~100 ESI MIRA applications won’t be reviewed in a timely manner… One of the many postponed meetings now, but it just breaks my heart and makes me feel super bad for all the junior faculty out there. What a shame.
9 months ago
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In some positive news - truly honored to be named a 2025 ASBMB Fellow. Humbled to be a part of this amazing cohort of researchers. -ok
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ASBMB names 2025 fellows
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.
https://www.asbmb.org/asbmb-today/people/021725/asbmb-names-2025-fellows
9 months ago
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reposted by
Khalimonchuk Lab
Jonathan Friedman
9 months ago
Some good news (and fresh science) to break things up! Our work identifying a subpopulation of compositionally unique mitochondria that localize to filopodia and promote cell migration is now
@currentbiology.bsky.social
! Led by fantastic
@madeleinempavey.bsky.social
!
www.cell.com/current-biol...
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Compositionally unique mitochondria in filopodia support cellular migration
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...
https://www.cell.com/current-biology/fulltext/S0960-9822(25)00125-3
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Dual regulation of mitochondrial fusion by Parkin–PINK1 and OMA1
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Dual regulation of mitochondrial fusion by Parkin–PINK1 and OMA1 - Nature
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, ...
https://www.nature.com/articles/s41586-025-08590-2
9 months ago
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HypoxyStat, a small-molecule form of hypoxia therapy that increases oxygen-hemoglobin affinity
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HypoxyStat, a small-molecule form of hypoxia therapy that increases oxygen-hemoglobin affinity
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.
https://www.cell.com/cell/fulltext/S0092-8674(25)00098-4?_returnURL=https%3A%2F%2Flinkinghub.elsevier.com%2Fretrieve%2Fpii%2FS0092867425000984%3Fshowall%3Dtrue
10 months ago
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reposted by
Khalimonchuk Lab
Osman Lab
10 months ago
🚀 Excited to share our latest work on Mrx6 and mtDNA copy number regulation, now available on
@biorxivpreprint.bsky.social
! We uncover how Mrx6 and Mam33 interact with the Lon protease Pim1 to control substrate degradation. 🔬 Check it out here:
www.biorxiv.org/content/10.1...
#mitochondria
#mtDNA
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Mrx6 binds the Lon protease Pim1 N-terminal domain to confer selective substrate specificity and regulate mtDNA copy number
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 ...
https://www.biorxiv.org/content/10.1101/2025.02.14.638270v1
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reposted by
Khalimonchuk Lab
Carl T. Bergstrom
10 months ago
1. Today the NIH director issued a new directive slashing overhead rates to 15%. I want to provide some context on what that means and why it matters.
grants.nih.gov/grants/guide...
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NOT-OD-25-068: Supplemental Guidance to the 2024 NIH Grants Policy Statement: Indirect Cost Rates
NIH Funding Opportunities and Notices in the NIH Guide for Grants and Contracts: Supplemental Guidance to the 2024 NIH Grants Policy Statement: Indirect Cost Rates NOT-OD-25-068. OD
https://grants.nih.gov/grants/guide/notice-files/NOT-OD-25-068.html
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reposted by
Khalimonchuk Lab
Mary Munson
10 months ago
đź§ŞThrilled to share the latest research from the lab of my awesome
@umasschan.bsky.social
colleague Jessica Spinelli (who isn't on here yet) and collaborators:
www.cell.com/cell/fulltex...
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Rhodoquinone carries electrons in the mammalian electron transport chain
Valeros et al. identify rhodoquinone (RQ), an electron carrier present in mammalian mitochondria, that can deliver electrons to fumarate instead of O2 as the terminal electron acceptor. The authors de...
https://www.cell.com/cell/fulltext/S0092-8674(24)01420-X
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Serine phosphorylation facilitates protein degradation by the human mitochondrial ClpXP protease
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Serine phosphorylation facilitates protein degradation by the human mitochondrial ClpXP protease | PNAS
ClpXP is a two-component mitochondrial matrix protease. The caseinolytic mitochondrial matrix peptidase chaperone subunit X (ClpX) recognizes and t...
https://www.pnas.org/doi/10.1073/pnas.2422447122
10 months ago
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An avoidance segment resolves a lethal nuclear–mitochondrial targeting conflict during ribosome assembly
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An avoidance segment resolves a lethal nuclear–mitochondrial targeting conflict during ribosome assembly - Nature Cell Biology
Oborská-Oplová et al. report a conserved mitochondrial avoidance segment in the cytosolic ribosomal protein uS5 that prevents mistargeting of uS5 to the mitochondrial matrix and ensures cytosolic ribo...
https://www.nature.com/articles/s41556-024-01588-4
10 months ago
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Mitochondria-plasma membrane contact sites regulate the ER-mitochondria encounter
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Mitochondria-plasma membrane contact sites regulate the ER-mitochondria encounter structure
Cells form multiple, molecularly distinct membrane contact sites (MCSs) between organelles. Despite knowing the molecular identity of several of these complexes, little is known about how MCSs are coo...
https://journals.biologists.com/jcs/article/doi/10.1242/jcs.263685/365483/Mitochondria-plasma-membrane-contact-sites
10 months ago
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reposted by
Khalimonchuk Lab
Christian Frezza
10 months ago
The mitochondrial DNAJC co-chaperone TCAIM reduces α-ketoglutarate dehydrogenase protein levels to regulate metabolism: Molecular Cell
www.cell.com/molecular-ce...
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The mitochondrial DNAJC co-chaperone TCAIM reduces α-ketoglutarate dehydrogenase protein levels to regulate metabolism
Wang et al. reveal TCAIM as a DNAJC co-chaperone that specifically binds OGDH, reducing its protein levels via mtHSP70 and LONP1. Departing from the classical chaperone role in protein folding, this r...
https://www.cell.com/molecular-cell/fulltext/S1097-2765%2825%2900036-X
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reposted by
Khalimonchuk Lab
Jakob Farnung
10 months ago
🎉Super excited to share our story on how the substrate receptor FBXO31 functions as a quality control factor by recognizing amides. This has been an amazing collaboration between Bode lab and
@jcornlab.bsky.social
. Special shutout goes to
@matthiasmuhar.bsky.social
www.nature.com/articles/s41...
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C-terminal amides mark proteins for degradation via SCF–FBXO31 - Nature
SCF–FBXO31 scans proteins for C-terminal amidation and marks them for subsequent proteasomal degradation.
https://www.nature.com/articles/s41586-024-08475-w
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www.nature.com/articles/s41...
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SLC25A38 is required for mitochondrial pyridoxal 5’-phosphate (PLP) accumulation - Nature Communications
Pyridoxal 5’-phosphate (PLP, vitamin B6) is crucial for various metabolic processes. Here, the authors identified SLC25A38 as a key regulator of mitochondrial PLP levels using a genome-wide CRISPRi sc...
https://www.nature.com/articles/s41467-025-56130-3
10 months ago
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www.embopress.org/doi/full/10....
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MTCH2 controls energy demand and expenditure to fuel anabolism during adipogenesis | The EMBO Journal
imageimageThe balance between cellular energy demand, expenditure, and anabolic processes to meet cells fundamental requirements remains debated. Using integrated temporal metabolomics and lipidomics,...
https://www.embopress.org/doi/full/10.1038/s44318-024-00335-7
11 months ago
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www.life-science-alliance.org/content/8/3/...
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COA5 has an essential role in the early stage of mitochondrial complex IV assembly
Pathogenic variants in cytochrome c oxidase assembly factor 5 (COA5), a proposed complex IV (CIV) assembly factor, have been shown to cause clinical mitochondrial disease with two siblings affected by...
https://www.life-science-alliance.org/content/8/3/e202403013
11 months ago
0
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www.nature.com/articles/s41...
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Triacylglycerol mobilization underpins mitochondrial stress recovery - Nature Cell Biology
Baker et al. show that mitochondrial stress recovery requires mobilization of lipid droplet triacylglycerol stores to facilitate cardiolipin biosynthesis and mitochondrial biogenesis.
https://www.nature.com/articles/s41556-024-01586-6
11 months ago
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www.nature.com/articles/s41...
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Mitochondrial YME1L1 governs unoccupied protein translocase channels - Nature Cell Biology
Hsu et al. show that mitochondrial import blockage stress activates the ATP-dependent protease YME1L1, which degrades mitochondrial presequence translocase TIM23 subunits to promote cell growth.
https://www.nature.com/articles/s41556-024-01571-z
11 months ago
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www.embopress.org/doi/full/10....
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Dbi1 is an oxidoreductase and an assembly chaperone for mitochondrial inner membrane proteins | EMBO reports
imageimageThis study identifies a pathway for oxidation and assembly of mitochondrial inner membrane proteins. Dbi1 is a conserved mitochondrial inner membrane protein that interacts with the unassem...
https://www.embopress.org/doi/full/10.1038/s44319-024-00349-6
11 months ago
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