A5104685287- Amyotrophic Lateral Sclerosis Research
- Neurogenetic and Muscular Disorders Research
- 3D Surveying and Cultural Heritage
- Histone Deacetylase Inhibitors Research
- RNA Research and Splicing
- Neurological diseases and metabolism
- Pluripotent Stem Cells Research
- Remote Sensing and LiDAR Applications
- Robotics and Sensor-Based Localization
- Prion Diseases and Protein Misfolding
- 3D Modeling in Geospatial Applications
- Plant-based Medicinal Research
- Muscle Physiology and Disorders
- biodegradable polymer synthesis and properties
- Signaling Pathways in Disease
- Cancer therapeutics and mechanisms
- Polyomavirus and related diseases
University Medical Center
2024
University of Rostock
2013-2023
TU Dresden
2015-2019
German Center for Neurodegenerative Diseases
2019
University of Florida
2019
Columbus Oncology and Hematology Associates
2019
Amyotrophic lateral sclerosis (ALS) is the most frequent motor neuron disease. Cytoplasmic fused in sarcoma (FUS) aggregates are pathological hallmarks of FUS-ALS. Proper shuttling between nucleus and cytoplasm essential for physiological cell function. However, initial event pathophysiology FUS-ALS remains enigmatic. Using human induced pluripotent stem (hiPSCs)-derived neurons (MNs), we show that impairment poly(ADP-ribose) polymerase (PARP)-dependent DNA damage response (DDR) signaling...
Despite decades of research on amyotrophic lateral sclerosis (ALS), there is only one approved drug, which minimally extends patient survival. Here, we investigated pathophysiological mechanisms underlying ALS using motor neurons (MNs) differentiated from induced pluripotent stem cells (iPSCs) derived patients carrying mutations in FUS or SOD1. Patient-derived MNs were less active and excitable compared to healthy controls, due reduced Na(+) /K(+) ratios both groups accompanied by elevated...
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder causing progressive loss of motor neurons. Mutations in Fused sarcoma (FUS) leading to its cytoplasmic mislocalization cause subset ALS. Under stress, mutant FUS localizes stress granules (SGs)-cytoplasmic condensates composed RNA and various proteins. Aberrant dynamics SGs linked the pathology Here, using neurons (MNs) derived from human induced pluripotent stem cells, we show that, FUS, MN disturbed. Additionally,...
Abstract Objective Mutations in Fused Sarcoma ( FUS or TLS ) are the fourth most prevalent Western European familial amyotrophic lateral sclerosis (ALS) populations and have been associated with causing both early very late disease onset. aggregation, DNA repair deficiency, genomic instability contributors to pathophysiology of FUS‐ALS, but their clinical significance per se influence on variability yet be sufficiently investigated. The aim this study was analyze genotype–phenotype...
Abstract Neurodegenerative diseases pose a complex field with various neuronal subtypes and distinct differentially affected intra-neuronal compartments. Modelling of neurodegeneration requires faithful in vitro separation axons dendrites, their distal proximal compartments as well organelle tracking defined retrograde versus anterograde directionality. We use microfluidic chambers to achieve compartmentalization established high throughput live imaging at standardized axonal readout sites...
Abstract. The main focus of the paper is a comparative study in which we have investigated, whether automatically generated digital surface models (DSM) obtained from unmanned aerial systems (UAS) imagery are comparable with DSM terrestrial laser scanning (TLS). research conducted at pilot dike for coastal engineering. effort and achievable accuracy both DSMs compared. error budgets these two methods investigated each case compared against other.
FUS (fused in sarcoma) plays a key role several steps of RNA metabolism, and dominant mutations this protein are associated with neurodegenerative diseases. Here, we show that is component the cellular response to topoisomerase I (TOP1)–induced DNA breakage; relocalising nucleolus polymerase II (Pol II) stalling at sites TOP1-induced breaks. This relocalisation rapid dynamic, reversing following removal breaks coinciding recovery global transcription. Importantly, breakage increased binding...
Intronic hexanucleotide repeat expansions (HREs) in C9ORF72 are the most frequent genetic cause of amyotrophic lateral sclerosis, a devastating, incurable motoneuron (MN) disease. The mechanism by which HREs trigger pathogenesis remains elusive. discovery repeat-associated non-ATG (RAN) translation dipeptide proteins (DPRs) from along with reduced exonic expression suggests gain toxic functions (GOFs) through DPRs versus loss (LOFs). Through multiparametric high-content (HC) live profiling...
Amyotropic lateral sclerosis (ALS) is a lethally progressive and irreversible neurodegenerative disease marked by apparent death of motor neurons present in the spinal cord, brain stem cortex. While more gene mutants being established for genetic ALS, vast majority suffer from sporadic ALS (>90%). It has been challenging, thus, to model which one reason why underlying pathophysiology remains elusive stalled development therapeutic strategies this neuron disease. To further unravel these...
Amyotrophic lateral sclerosis (ALS) is a fatal disorder characterized by degeneration of the upper and lower motor neuron. Among at least 25 known genes associated with familial (hereditary) sporadic ALS, mutations in fused-in-sarcoma (FUS) superoxide dismutase 1 (SOD1) have been extensively investigated past years, emphasis on altered excitability affected neurons. Recently, we reported hypoexcitability increased cell death FUS/SOD1-ALS-induced pluripotent stem cell-derived neuron model,...
Recent research has demonstrated significant aberrant activation of the innate immune system in ALS model systems due to mutations SOD1, TARDBP and C9orf72 through stimulation TBK1-IRF3 pathway. This pathway can be activated, for example, by cGAS-STING-dependent sensing cytosolic DNA that accumulates as a result chronic damage defective mitochondria, both which have been identified early pathology FUS-ALS spinal motor neurons (sMNs). Therefore, we analysed pathways isogenic non-isogenic...
Abstract. UAS become a very valuable tool for coastal morphology. Not only mapping but also change detection and better understanding of processes along across the shore. This contribution investigates possibilities to determine water depth in clear shallow waters by means so called "photo bathymetry". From results several test flights it became that three factors influence ability accuracy bathymetric sea floor measurements. Firstly, weather conditions. Sunny is not always good. Due high...