Alessandro Prigione

Congrats Christian and team!!

An atlas of mitochondrial ATP synthase activity across the lifespan Mitochondrial dysfunction and declining energy production are hallmarks of aging, yet we lack a comprehensive systems-level view of ATP synthase (Comp…

An atlas of mitochondrial ATP synthase activity across the lifespan #mitochondria
www.sciencedirect.com/science/arti...

Brain organoid models of Huntington's disease shift the focus towards neurodevelopment Summary: Increasing evidence indicates neurodevelopmental aspects in Huntington's disease. Early interventions promoting healthy brain development and mitochondrial fitness might lead to prevention or...

Our latest perspective is out in Dis Model Mech (@biologist.bsky.social)!
We discuss how brain organoids highlight the impact of Huntington's disease on brain development. We suggest takling mitochondrial fitness early on a as a potential treatment strategy.

doi.org/10.1242/dmm....

Fantastic discovery! Congratulations!

Cool! Congratulations Carmelo and team!!

Israel has committed genocide in Gaza, UN commission of inquiry says The panel finds that four of the five genocidal acts defined under international law have been carried out against Palestinians during the war.

Israel has committed genocide in Gaza, UN commission of inquiry says www.bbc.com/news/article...

Cells are swapping their mitochondria. What does this mean for our health? Researchers are studying why the energy factories are moving between cells and whether the process can be harnessed to treat cancer and other diseases.

Researchers are studying why mitochondria are moving between cells and whether the process can be harnessed to treat cancer and other diseases

go.nature.com/4fYoEwG

Complex genetic variation in nearly complete human genomes - Nature Using sequencing and haplotype-resolved assembly of 65 diverse human genomes, complex regions including the major histocompatibility complex and centromeres are analysed.

Two papers in today's issue of @nature.com ‬: 1) we assemble 65 genomes to near completion, including centromeres and the MHC. tinyurl.com/3huhax6w. 2) we sequence 1,019 genomes from the 1kGP with long reads, revealing SVs down to low allele frequencies tinyurl.com/wbx3we9x.

Neuronal branching in stem cell models of mitochondrial and neurological diseases Abstract. Neuronal branching, the extension and arborization of neurites, is critical for establishing and maintaining functional neural circuits. Emerging

Our latest review is out in STEM CELLS!
We discuss the role of mitochondria in neuronal branching and how iPSC models in 2D and 3D can be used dissect the impact of neuronal branching defects in mitochondrial disorders.

academic.oup.com/stmcls/advan...

Picture of Alessandro Prigione featuring his photo and a short biography. Alessandro Prigione will be speaking at Euromit congress in 2026 in Angers, France on Wednesday, June 3rd during the session entitled Innovative technologies. Alessandro Prigione is a professor at the Heinrich Heine University in Düsseldorf, Germany. His group uses patient-derived iPSCs to generate cerebral cells and organoids to study how mitochondrial dysfunction leads to neurological disorders such as Leigh syndrome. He uses advanced stem cell technologies, genome editing, and high-throughput drug screenings to understand disease mechanisms and identify potential therapies.

🎙️ Focus Speakers – Alessandro Prigione

👀 Curious to know who will be speaking at #euromit2026 ?
✨ Today, we’re introducing A. Prigione @aleprigio.bsky.social who will be taking the stage at the congress.
#euromit2026 #MitochondrialDisease #mtDNA #mitochondria #angers
@ua-recherche.bsky.social

Looking forward to Euromit 2026!!

A mitochondrial disease model is generated and corrected using engineered base editors in rat zygotes - Nature Biotechnology A mitochondrial disease is modeled using engineered base editors in rats.

mtDNA editing!

Thank you Antonio! We think it goes through PRKG1 which in turn may act on calcium homeostasis on one hand and on neurite outgrowth on the other. But still it is likely pleiotropic, so more mechanisms could be in place

Pluripotent stem cell-based drug discovery uncovers sildenafil as a treatment for mitochondrial disease Mitochondrial disease encompasses inherited disorders affecting mitochondrial function. A severe and untreatable form of mitochondrial disease is Leigh syndrome (LS) causing psychomotor regression and...

Exciting news! Our most important work is out as preprint!

We suggest sildenafil as a treatment for mitochondrial disease Leigh syndrome, from stem and organoid model to mice and off-label use.

Next step is the clinical trial starting early next year!

www.medrxiv.org/content/10.1...

Spatial transcriptomics reveals human cortical layer and area specification - Nature Multiplexed error-robust fluorescence in situ hybridization (MERFISH) together with deep-learning-based nucleus segmentation enabled the construction of a highly detailed and informative spatially resolved single-cell atlas of human fetal cortical development.

Nature research paper: Spatial transcriptomics reveals human cortical layer and area specification

https://go.nature.com/3GUX8Tm

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

www.nature.com/articles/s41...

A human-specific enhancer fine-tunes radial glia potency and corticogenesis - Nature HARE5, a human accelerated region enhancer, modulates cortical development by influencing neural progenitor cell behaviour, leading to an enlarged neocortex with increased functional independence betw...

Thrilled to share our latest study out in @natureportfolio.nature.com led by the fantastically talented Jing Liu. Our study provides insight into a long standing question in biology: What molecular features make us uniquely human and how do these function? www.nature.com/articles/s41...

I'm thrilled to be co-organizing the very first EMBO Workshop bridging mitochondria and brain research - a powerhouse of science, ideas, and amazing speakers! 🧠🔬

📅 registration closes MAY 30th, so don’t wait to snag your spot!

Stay tuned - more updates about the topics and speakers coming soon! 🎙️

Redefining CNS immune privilege - Nature Reviews Immunology In this Perspective, Smyth and Kipnis reappraise the concept of immune privilege in the central nervous system. Although immune privilege was originally thought to involve isolation of the central ner...

Our perspective in @natrevimmunol.nature.com on new concepts in neuroimmune interactions that have challenged the old dogma that immune privilege is a result of the isolation of the nervous and immune systems.

@jonykipnis.bsky.social
www.nature.com/articles/s41...

Vascular network-inspired diffusible scaffolds for engineering functional midbrain organoids Guo and colleagues developed vascular network-inspired diffusible (VID) scaffolds for engineering midbrain organoids, providing a versatile technology for organoid development and therapeutic innovation.

Vascular network-inspired diffusible scaffolds for engineering functional midbrain organoids

Stem cells as role models for reprogramming and repair Stem cells are a promising source for cellular therapies across many diseases and tissues. Their inherent ability to differentiate into other cell types has been the focus of investigation over decade...

After decades of research on stem cells and their reprogramming, we're now seeing extensive clinical trial assessment, some with very encouraging results. This is a new @science.org outstanding review of the biology and ongoing research efforts
www.science.org/doi/10.1126/...

Regional differences in progenitor metabolism shape brain growth during development Brain-wide birthdate mapping reveals that while hindbrain neurogenesis is transient, forebrain neurogenesis is temporally sustained through spatiotemporal regulation of progenitor mitochondrial functi...

Great study by @nataliabaumann.bsky.social @djabaudon.bsky.social & collaborators, showing how regional differences in progenitor metabolism shape brain growth during development
@cp-cell.bsky.social

www.cell.com/cell/abstrac...

Cellular interplay in brain organoids: Connecting cell-autonomous and non-cell-autonomous mechanisms in neurodevelopmental disease The field of brain organoids has experienced a period of rapid and transformative growth, enabling researchers to investigate complex human biological…

www.sciencedirect.com/science/arti...

Retromer promotes the lysosomal turnover of mtDNA Lysosomal uptake of mtDNA controls mitochondrial quality.

Excited to share our latest paper in Science Advances! We finally dissected a new pathway for mitochondrial quality control upon mtDNA replication stress! Let’s start a short thread! 🧵

www.science.org/doi/10.1126/...

Congratulations David! Amazing work!

Mitochondria in the Nervous System The brain is a high energy-demanding tissue with an heterogenous cellular composition. Most of the energy consumed is used for maintenance of neuronal synaptic transmission, and neurons almost exclus…

Thrilled to announce that the registration for the EMBO workshop „Mitochondria in the Nervous System“ is now open.
Join us from Sep 29th to Oct 2nd in beautiful Engelberg, Switzerland!
meetings.embo.org/event/25-mit...

A call to action for deciphering genetic variants in human pluripotent stem cells for cell therapy Human pluripotent stem cell (hPSC)-based therapies offer promise but pose potential risks due to culture-acquired genetic variants, some of which have been linked with cancer. An international worksho...

www.cell.com/cell-stem-ce...

Honored to be part of this landmark forum article @cp-cellstemcell.bsky.social outlining a roadmap for genomic safety in human pluripotent stem cell therapies. Thankful to Ivana Barbaric for leadership and to all coauthors for great efforts.

kwnsfk27.r.eu-west-1.awstrack.me/L0/https://a...

Science under siege: protecting scientific progress in turbulent times As Editors-in-Chief of The Company of Biologists' journals, we have been watching with growing concern the policy changes in the United States of America (USA) and the challenges that these are creati...

"we have been watching with growing concern policy changes in the US...The scale of disruption to science is unprecedented...We are concerned with policies that threaten censorship & removal of public data...To our colleagues in the US, we stand with you" journals.biologists.com/jcs/article/...

Natural History of Patients With Mitochondrial ATPase Deficiency Due to Pathogenic Variants of MT-ATP6 and MT-ATP8 | Neurology Background and ObjectivesThe mitochondrial DNA (mtDNA) genes MT-ATP6 and MT-ATP8 encode for subunits α and 8 (A6L) of the adenosine triphosphate synthase complex. Pathogenetic variants in MT-ATP6/8 ca...

Wonderful to see this out!Thanks to all clinicians for sharing their data. By looking at the natural history of Leigh syndrome patients with mitochondrial mutations MT-ATP6 and MT-ATP8 we can learn key aspects to monitor for future clinical trials.

www.neurology.org/doi/10.1212/...