We study the organization of the mitochondrial genome, mitochondrial gene expression, mitochondria i
We study the organization of the mitochondrial genome, mitochondrial gene expression, mitochondria in ageing and mitochondria in neurodegenerative diseases
05/07/2021
replication
The mitochondrial single-stranded DNA binding protein is essential for initiation of mtDNA replication We report a role for the mitochondrial single-stranded DNA binding protein (mtSSB) in regulating mitochondrial DNA (mtDNA) replication initiation in mammalian mitochondria. Transcription from the light-strand promoter (LSP) is required both for gene expression and for generating the RNA primers need...
16/12/2020
Great new study from our lab!
Small-molecule inhibitors of human mitochondrial DNA transcription Inhibitors of mitochondrial transcription that target human mitochondrial RNA polymerase provide a chemical biology tool for studying the role of mitochondrial DNA expression in a wide range of pathologies.
05/11/2019
....read about new tool to study mitoribosomes in vivo. Congrats Jakob Busch et al!!!
MitoRibo-Tag Mice Provide a Tool for In Vivo Studies of Mitoribosome Composition Busch et al. generated MitoRibo-Tag mice to study mitoribosome composition in vivo in different tissues. Proteomics of MitoRibo-Tag mice with defective assembly identified PUSL1 as a mitoribosome-interacting protein required for efficient mitochondrial translation. MitoRibo-Tag mice provide a tool...
11/06/2019
Read about latest research from our lab! Eduardo Silva Ramos et al.
Check also the preview in the same issue https://journals.plos.org/plosgenetics/article?id=10.1371/journal.pgen.1008140
Mitochondrial fusion is required for regulation of mitochondrial DNA replication Author summary Mammalian mitochondria contain multiple copies of the mitochondrial genome (mtDNA), which encodes genes that are essential for the oxidative phosphorylation system. An important feature of mtDNA is that it is evenly distributed throughout the mitochondrial network. Dynamin-related GTP...
26/09/2018
mtDNA/ fruit flies/ ageing new paper from our lab!!!!!
Mutations of mitochondrial DNA are not major contributors to aging of fruit flies Mutations of mtDNA accumulate in aging humans and other mammals to cause mitochondrial dysfunction in a subset of cells in various tissues. Furthermore, experimental induction of mtDNA mutations causes a premature aging syndrome in the mouse. To study if mitochondrial dysfunction is universally invo...
24/09/2018
A new collaborative publication with Moraes lab and Stewart lab is now out! Read how to decrease the level of mitochondrial DNA molecules carrying a pathogenic mutation in the newest volume of Nature Medicine. Check also the accompanying paper from Gammage et al.
https://www.nature.com/articles/s41591-018-0166-8
https://www.nature.com/articles/s41591-018-0165-9
MitoTALEN reduces mutant mtDNA load and restores tRNAAla levels in a mouse model of heteroplasmic mtDNA mutation Mitochondrial-targeted TALENs reduce mutation burden and correct biochemical defects in a mouse model of mitochondrial disease.
19/06/2018
Want to work with mitochondrial genetics? We are now hiring 1-2 postdocs in our lab in Stockholm, Sweden. Apply before 25th of June!
1-2 Postdoctoral researchers in Mitochondrial Genetics : Stockholm, Sweden : Naturejobs 1-2 Postdoctoral researchers in Mitochondrial Genetics - Karolinska Institutet (KI) are looking for a Other in Stockholm, Sweden. Salary: unspecified, Hours: Full-time, Type: Contract
What do you think is inducing more mitochondrial DNA mutations: Replication errors or oxidative damage? We have now pretty good evidence showing it is not the latter. Now in Nucleic Acids Research!🎉
19/05/2018
Not from our department but hey, same institution and it's about mitochondria 😎
Are you feeling blue due to the low sensitivity of your COX/SDH staining? There is now a new staining in town, NBTx! 🎨
Use it to visualize and quantify mitochondrial COX defects in your favourite model organism.
A novel histochemistry assay to assess and quantify focal cytochrome c oxidase deficiency. - PubMed - NCBI J Pathol. 2018 Apr 16. doi: 10.1002/path.5084. [Epub ahead of print]
06/04/2018
A new collaborative publication with Aleksandra Filipovska and Oliver Rackham is now out! It is open access so if you are curious what pentatricopeptide repeat domain protein 1 ( PTCD1) is doing in mitochondria, now is your chance to find out.
PTCD1 Is Required for 16S rRNA Maturation Complex Stability and Mitochondrial Ribosome Assembly Perks et al. engineered intron-exon boundaries using CRISPR/Cas9 to conditionally knock out Ptcd1 in mice. The RNA-binding protein PTCD1 is essential for heart function and regulates the stability and maturation of the 16S rRNA. PTCD1 is required for mitoribosome biogenesis, mitochondrial function,....
24/03/2018
☀️Spring is coming and so are the publications! 🐣In humans, mutations in mitochondrial genome maintenance exonuclease 1 (MGME1) lead to a disease. In our newest publication we characterize MGME1 knockout mouse model. What are the differences and similarities? It's Open Access so go find out!
Mice lacking the mitochondrial exonuclease MGME1 accumulate mtDNA deletions without developing progeria It has been debated whether premature ageing in mitochondrial DNA mutator mice is driven by point mutations or deletions of mtDNA. Matic et al generate Mgme1 knockout mice and show here that these mice have tissue-specific replication stalling and accumulate deleted mtDNA, without developing progeri...