Khalimonchuk Lab
@mitocrue.bsky.social
Semper vestigans | All things mitochondria
https://biochem.unl.edu/oleh-khalimonchuk-lab/
https://biochem.unl.edu/oleh-khalimonchuk-lab/
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-...
www.cell.com
October 22, 2025 at 12:54 AM
Delayed protein translocation protects mitochondria against toxic CAT-tailed proteins
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 ...
www.nature.com
October 14, 2025 at 5:00 PM
MAPL regulates gasdermin-mediated release of mtDNA from lysosomes to drive pyroptotic cell death
Reposted by Khalimonchuk Lab
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
October 9, 2025 at 8:47 PM
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.
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...
www.nature.com
August 28, 2025 at 5:30 PM
Dynamic TOM–TIM23 supercomplex directs mitochondrial protein translocation and sorting.
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…
www.sciencedirect.com
August 27, 2025 at 5:46 PM
Proximity-specific ribosome profiling reveals the logic of localized mitochondrial translation
Super-resolution microscopy of mitochondrial mRNAs
idp.nature.com
August 15, 2025 at 9:45 PM
Super-resolution microscopy of mitochondrial mRNAs
Mitochondrial origins of the pressure to sleep
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.
www.nature.com
July 17, 2025 at 1:51 AM
Mitochondrial origins of the pressure to sleep
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
July 8, 2025 at 8:21 PM
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
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 ...
www.cell.com
June 26, 2025 at 11:45 PM
Interaction with AK2A links AIFM1 to cellular energy metabolism
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) ...
www.nature.com
June 23, 2025 at 5:31 PM
ACAD10 and ACAD11 enable mammalian 4-hydroxy acid lipid catabolism
🔥🔥 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...
www.nature.com
June 16, 2025 at 7:43 PM
🔥🔥 Structural mechanism of mitochondrial membrane remodelling by human OPA1
Excited to visit LMU!
📣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
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
June 1, 2025 at 10:25 PM
Excited to visit LMU!
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...
www.embopress.org
May 24, 2025 at 6:40 PM
Mechanistic insights into Bcs1-mediated mitochondrial membrane translocation of the folded Rieske protein
Reposted by Khalimonchuk Lab
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
May 23, 2025 at 7:23 PM
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
Reposted by Khalimonchuk Lab
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.
We present the MINDNet: Proximity Proteomics revealing the nanodomain interactome in the mitochondrial inner membrane, done by the talented Yulia and team.
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
May 21, 2025 at 6:29 PM
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.
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.
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...
www.nature.com
May 15, 2025 at 2:11 AM
Molecular machineries shaping the mitochondrial inner membrane.
Reposted by Khalimonchuk Lab
Fmp30 is a phosphatidylinositol hydrolase that regulates CoQ biosynthesis https://www.biorxiv.org/content/10.1101/2025.05.01.651778v1
May 2, 2025 at 4:46 PM
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...
www.biorxiv.org
April 25, 2025 at 6:15 PM
Structures of human organellar SPFH protein complexes
Molecular basis of pyruvate transport and inhibition of the human mitochondrial pyruvate carrier
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.
www.science.org
April 18, 2025 at 11:20 PM
Molecular basis of pyruvate transport and inhibition of the human mitochondrial pyruvate carrier
Small molecules restore mutant mitochondrial DNA polymerase activity
https://www.nature.com/articles/s41586-025-08856-9
t.co
April 9, 2025 at 5:25 PM
Small molecules restore mutant mitochondrial DNA polymerase activity
Running a genetic stop sign accelerates oxygen metabolism and energy production in horses
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...
www.science.org
March 30, 2025 at 7:55 PM
Running a genetic stop sign accelerates oxygen metabolism and energy production in horses
Oxidation of retromer complex controls mitochondrial translation
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...
www.nature.com
March 26, 2025 at 6:47 PM
Oxidation of retromer complex controls mitochondrial translation
A splendid molecular factory: De- and reconstruction of the mammalian respiratory chain
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...
www.pnas.org
March 23, 2025 at 1:03 PM
A splendid molecular factory: De- and reconstruction of the mammalian respiratory chain
In situ architecture of the human prohibitin complex
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...
www.nature.com
March 22, 2025 at 1:10 AM
In situ architecture of the human prohibitin complex
In-cell architecture of the mitochondrial respiratory chain
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...
www.science.org
March 21, 2025 at 4:53 PM
In-cell architecture of the mitochondrial respiratory chain