A5082156932- Autophagy in Disease and Therapy
- Muscle Physiology and Disorders
- Ubiquitin and proteasome pathways
- Genetic Syndromes and Imprinting
- Cell death mechanisms and regulation
- Cardiomyopathy and Myosin Studies
- ATP Synthase and ATPases Research
- Endoplasmic Reticulum Stress and Disease
- RNA modifications and cancer
- Sirtuins and Resveratrol in Medicine
- Toxoplasma gondii Research Studies
- Mitochondrial Function and Pathology
- Genetic Neurodegenerative Diseases
- Studies on Chitinases and Chitosanases
University of Freiburg
2020-2025
University of Basel
2017-2019
Mitochondria are key organelles for cellular energetics, metabolism, signaling, and quality control have been linked to various diseases. Different views exist on the composition of human mitochondrial proteome. We classified >8,000 proteins in preparations cells defined a high-confidence proteome >1,100 (MitoCoP). identified interactors translocases, respiratory chain, ATP synthase assembly factors. The abundance MitoCoP covers six orders magnitude amounts 7% with chaperones HSP60-HSP10...
Abstract Loss of innervation skeletal muscle is a determinant event in several diseases. Although effectors have been identified, the pathways controlling integrated response to denervation remain largely unknown. Here, we demonstrate that PKB/Akt and mTORC1 play important roles regulating homeostasis maintaining neuromuscular endplates after nerve injury. To allow dynamic changes autophagy, activation must be tightly balanced following denervation. Acutely activating or inhibiting impairs...
Myotonic dystrophy type I (DM1) is a disabling multisystemic disease that predominantly affects skeletal muscle. It caused by expanded CTG repeats in the 3'-UTR of dystrophia myotonica protein kinase (DMPK) gene. RNA hairpins formed elongated DMPK transcripts sequester RNA-binding proteins, leading to mis-splicing numerous pre-mRNAs. Here, we have investigated whether DM1-associated muscle pathology related deregulation central metabolic pathways, which may identify potential therapeutic...
ABSTRACT The formation of multi-nucleated muscle fibers from progenitors requires the fine-tuned and coordinated regulation proliferation, differentiation fusion, both during development after injury in adult. Although some key factors that are involved different steps well known, how intracellular signals integrated is largely unknown. Here, we investigated role cell-growth regulator mTOR by eliminating essential components complexes 1 (mTORC1) 2 (mTORC2) mouse progenitors. We show...
Mitophagy is a specific type of autophagy responsible for the selective elimination dysfunctional or superfluous mitochondria, ensuring maintenance mitochondrial quality control. The initiation mitophagy coordinated by ULK1 kinase complex, which engages receptors via its FIP200 subunit. Whether performs additional functions in subsequent later phases beyond this initial step and how regulation occurs, remains unclear. Our findings reveal that multiple phosphorylation events on differentially...