Khalimonchuk Lab

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

A splendid molecular factory: De- and reconstruction of the mammalian respiratory chain

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...

In situ architecture of the human prohibitin complex

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...

In-cell architecture of the mitochondrial respiratory chain

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...

🔥🔥 Structure of human PINK1 at a mitochondrial TOM-VDAC array

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 ...

Heterooligomerization drives structural plasticity of eukaryotic peroxiredoxins

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.

Engineering mtDNA deletions by reconstituting end joining in human mitochondria

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...

Structure of mitochondrial pyruvate carrier and its inhibition mechanism

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...

MARIGOLD and MitoCIAO, two searchable compendia to visualize and functionalize protein complexes during mitochondrial remodeling: Cell Metabolism www.cell.com/cell-metabol...