Research topic


Why are they worthy of their name?

Our aim is to decipher the role of mitochondria as main regulators of signaling transductions, especially in the case of OXPHOS dysfunction. When mitochondria experience stress or when dysfunction occurs, the organelle sends signals to the nucleus, which launches different types of adaptive responses. Transcription factors are activated and stimulate the expression of specific sets of genes, whose products enable the cell to adapt to the changes. We aim to further understand these largely unknown mechanisms that play a central role in determining the extent of tissue specific defect arising from the respiratory chain deficiency. The primary focus of our research is in deciphering the precise signaling cascade of the pathogenic mechanisms leading to mitochondrial diseases and ageing, with the ultimate goal of identifying new therapeutic targets and strategies.

The group mainly uses in vivo model systems: multiple transgenic mouse models and roundworm Caenorhabditis elegans to tackle specific questions of mitochondrial pathophysiology. The group relies on various molecular biology techniques to understand the complex signaling pathways, many of which are specifically developed to understand the mitochondrial physiology. To tackle complex molecular mechanisms of specific processes in details, we often turn to mammalian cell-based models and different biochemical approaches.



Mitochondria-originated redox signalling regulates KLF-1 to promote longevity in Caenorhabditis elegans.

FGF21 modulates mitochondrial stress response in cardiomyocytes only under mild mitochondrial dysfunction.

CLPP deficiency ameliorates neurodegeneration caused by impaired mitochondrial protein synthesis

Matthijs Hermeling defends his PhD thesis

On 21 January 2022 Matthijs Hermeling successfully defended his PhD Thesis

Adaptation to mitochondrial stress requires CHOP-directed tuning of ISR

Anastasia Rumyantseva defends her PhD thesis

On 19 Februar 2021 Anastasia Rumyantseva successfully defended her PhD Thesis

Karolina Szczepanowska, PhD becomes a group leader at the IMOL

Laboratory of Metabolic Quality Control, The International Institute of Molecular Mechanisms and Machines, Polish Academy of Sciences, Warsaw, Poland

Tune instead of destroy: How proteolysis keeps OXPHOS in shape

Identification of Putative Mitochondrial Protease Substrates

DARS2 is indispensable for Purkinje cell survival and protects against cerebellar ataxia

The Mouse Heart Mitochondria N Terminome Provides Insights into ClpXP-Mediated Proteolysis

Sarah Maciej defends her PhD thesis

On 26 June 2020 Sarah Maciej successfully defended her PhD Thesis

New insights into ClpXP-mediated proteolysis

Repair instead of renew in mammalian mitochondria

Sophie Kaspar defends her PhD thesis

On 13 February 2020 Sophie Kaspar successfully defended her PhD Thesis

Eduard Hofsetz defends his PhD thesis

On 29 November 2019 Eduard Hofsetz successfully defended his PhD Thesis

Detox pathway extends lifespan of the worm C. elegans


Metabolic alterations associated with CLPP-deficiency