Research topic

Mitochondria are essential organelles found in every eukaryotic cell, required to convert food into usable energy. The mitochondrial oxidative phosphorylation (OXPHOS), which produces the majority of cellular energy in the form of ATP, is controlled by two distinct genomes: the nuclear and the mitochondrial genome (mtDNA). Mutations in mitochondrial genes encoded by either genome could cause mitochondrial disorders, and have emerged as a key factor in a myriad of “common” diseases, including Parkinson’s and Alzheimer’s Disease, Type 2 Diabetes, and are strongly linked to the ageing process. Despite all this, it is surprising that our understanding of the mechanisms governing the mitochondrial gene expression, its reliance on the complex nature of dual genome control and associated pathologies remains superficial, with therapeutic interventions largely unexplored. Remarkably, mitochondria are now also viewed as main regulators of signal transduction. Within the last few years, multiple mitochondria-centric signalling mechanisms have been proposed, including release of multiple metabolites, and the scaffolding of signalling complexes on the outer mitochondrial membrane. It has also been shown that mitochondrial dysfunction causes induction of stress responses, bolstering the idea that mitochondria communicate their fitness to the rest of the cell. Studies in this area are not only of basic scientific interest, but may also provide new avenues towards treatment of mitochondrial dysfunction in a variety of human diseases and ageing.

The group mainly uses in vivo transgenic mouse models and also the roundworm Caenorhabditis elegans to tackle specific questions of mitochondrial pathophysiology. Many of the transgenic mice models are developed within the group. The group relies mainly on various molecular biology techniques to understand the complex signalling 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. As one of the main aims is to understand the consequences of energy depletion in cells and the organism as a whole, the lab has established many different bioenergetic approaches in vitro and in vivo, and this expertise is provided to the Cologne research community.



Welcome Nidhi!

Nidhi Nair joined the lab as new CGA PhD student.

Congratulations Hien!

On 30 May 2023 Hien Rozsivalova successfully defended her PhD Thesis!

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

Congratulations Matthijs!

On 21 January 2022 Matthijs Hermeling successfully defended his PhD Thesis

Adaptation to mitochondrial stress requires CHOP-directed tuning of ISR

Congratulations Anastasia!

On 19 Februar 2021 Anastasia Rumyantseva successfully defended her PhD Thesis

Congratulations Karolina!

KarolinaSzczepanowska, 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