A5089384868- Genetic Neurodegenerative Diseases
- Nerve injury and regeneration
- Peripheral Neuropathies and Disorders
- Hereditary Neurological Disorders
- Pain Mechanisms and Treatments
- Endoplasmic Reticulum Stress and Disease
- Ubiquitin and proteasome pathways
- Fibromyalgia and Chronic Fatigue Syndrome Research
- Botulinum Toxin and Related Neurological Disorders
- Toxin Mechanisms and Immunotoxins
- Autophagy in Disease and Therapy
- Multiple Myeloma Research and Treatments
- Phytochemistry and Biological Activities
- Phytochemicals and Antioxidant Activities
- Herbal Medicine Research Studies
- Bioactive natural compounds
- Acute Ischemic Stroke Management
- Multiple Sclerosis Research Studies
- Muscle Physiology and Disorders
- Pharmacological Effects of Natural Compounds
- Blood Coagulation and Thrombosis Mechanisms
- Atherosclerosis and Cardiovascular Diseases
- Polysaccharides and Plant Cell Walls
- Parkinson's Disease Mechanisms and Treatments
- Pediatric health and respiratory diseases
St. Josef-Hospital
2019-2025
Ruhr University Bochum
2016-2025
Lund University
2025
In inflammatory neuropathies, oxidative stress results in neuronal and Schwann cell (SC) death promoting early neurodegeneration clinical disability. Treatment with the short-chain fatty acid propionate showed a significant immunoregulatory neuroprotective effect multiple sclerosis patients. Similar effects have been described for patients chronic demyelinating polyneuropathy (CIDP). Therefore, cell’s survival dorsal root ganglia (DRG) outgrowth were evaluated vitro after treatment...
Abstract NEMO is a ubiquitin-binding protein which regulates canonical NF-κB pathway activation in innate immune signaling, cell death regulation and host-pathogen interactions. Here we identify an NF-κB-independent function of proteostasis by promoting autophagosomal clearance aggregates. NEMO-deficient cells accumulate misfolded proteins upon proteotoxic stress are vulnerable to challenges. Moreover, patient with mutation the NEMO-encoding IKBKG gene resulting defective binding linear...
The transgenic mouse model R6/2 exhibits Huntington's disease (HD)-like deficits and basic pathophysiological similarities. We also used the pheochromocytoma-12 (PC12)-cell-line-model to investigate effect of laquinimod on metabolic activity. Laquinimod is an orally administered immunomodulatory substance currently under development for treatment multiple sclerosis (MS) HD. As essential effect, increased levels BDNF were observed. Therefore, we investigated therapeutic efficacy in model,...
Abstract Background Only few studies describe the impact of nutritive factors on chronic inflammatory demyelinating polyneuropathy (CIDP), an disease peripheral nervous system. The active component chili pepper, capsaicin, is direct agonist transient receptor potential channel vanilloid subfamily member 1. Its anti-inflammatory effect in animal model experimental autoimmune neuritis (EAN) has been previously demonstrated. Methods In present study, we and anti-oxidative influence capsaicin...
Huntington's disease is a progressive, untreatable neurodegenerative disorder caused by mutation in the Huntingtin gene. Next to neurodegeneration, altered immune activation involved progression. Since central nervous system inflammation and dysfunction of cells are recognized as driving characteristics, immunomodulation might represent an additional therapeutic strategy. Short-chain fatty acids were known have immunomodulatory effects neuroinflammatory diseases, such multiple sclerosis. In...
Abstract Experimental autoimmune neuritis is a common animal model for acute human immune–mediated polyneuropathies. Although already established in 1955, number of pathophysiological mechanisms remain unknown. In this study, we extensively characterize experimental progression Lewis rats, including new insights into the integrity small nerve fibres, neuropathic pain and macrophage activation. Acute was induced with P253–78 peptide consequently investigated using gait analysis system CatWalk...
Abstract Activation of the kallikrein-kinin system promotes vascular leakage, inflammation, and neurodegeneration in ischemic stroke. Inhibition plasma kallikrein (PK) – a key component KKS acute phase stroke has been reported to reduce thrombosis, damage blood-brain barrier. However, role PK during recovery after cerebral ischemia is unknown. To this end, we evaluated effect subacute inhibition starting from day 3 on process transient middle artery occlusion (tMCAO). Our study demonstrated...
Proteasome inhibition with bortezomib has been reported to exert an immunomodulatory action in chronic autoimmune neuropathies. However, used for the treatment of multiple myeloma induces a painful toxic polyneuropathy at higher concentration. Therefore, we addressed this controversial effect and evaluated neurotoxic mode experimental neuritis. Bortezomib-induced neuropathy was investigated Lewis rats using von Frey hair test, electrophysiological, qPCR histological analyses sciatic nerve as...
ABSTRACT NEMO is a ubiquitin-binding protein which regulates canonical NF-κB pathway activation in innate immune signaling, cell death regulation and host-pathogen interactions. Here we identified an NF-κB-independent function of proteostasis by promoting autophagosomal clearance aggregates. NEMO-deficient cells accumulate misfolded proteins upon proteotoxic stress are vulnerable to challenges. Moreover, patient with mutation the gene resulting defective binding linear ubiquitin chains,...
The process of myelination is essential to enable rapid and sufficient signal transduction in the nervous system. In peripheral system, neurons Schwann cells engage a complex interaction control axons. Disturbances this breakdown myelin sheath are hallmarks inflammatory neuropathies occur secondarily neurodegenerative disorders. Here, we present coculture model dorsal root ganglion explants cells, which develops robust axons investigate study axon-Schwann cell interactions, evaluate...
Abstract So far, only a small number of medications are effective in progressive multiple sclerosis (MS). The sphingosine-1-phosphate-receptor (S1PR)-1,5 modulator siponimod, licensed for MS, is acting both on peripheral immune cells and the central nervous system (CNS). far it remains elusive, whether those effects related to neurotrophin brain derived neurotrophic factor (BDNF). We hypothesized that BDNF might be prerequisite reduce disease activity experimental autoimmune...
<h3>Introduction</h3> The transgenic mouse model R6/2 (141–157 CAG repeats) of Huntington’s disease (HD) recapitulates basic pathophysiological similarities HD and some its clinical symptoms. Laquinimod as an immunomodulatory orally substance has shown to downregulate astrocytic microglial activation which are common pathways in neurodegenerative diseases. <h3>Methods</h3> We investigated the therapeutic efficacy laquinimod treating animals with different concentrations (0.5/1.5/5/25 mg/kg...
The process of myelination is essential to enable rapid and sufficient signal transduction in the nervous system. In peripheral system, neurons Schwann cells engage a complex interaction control axons. Disturbances this breakdown myelin sheath are hallmarks inflammatory neuropathies occur secondarily neurodegenerative disorders. Here, we present coculture model dorsal root ganglion explants cells, which develops robust axons investigate study axon-Schwann cell interactions, evaluate...
To achieve effective treatment of hearing loss using cochlear implants the preservation spiral ganglion neurons (SGN) is a key goal. As hallmark neuronal cell death oxidative stress contributes to pathology deafness. Fumaric acid esters (FAEs) are known for their anti-oxidative and neuroprotective properties oral application dimethyl fumarate (DMF) already an approved multiple sclerosis. Hence, FAEs represent promising candidate SGN neuroprotection.
Zielsetzung Für den effektiven Einsatz von Cochlea Implantaten ist der dauerhafte Erhalt Spiralganglion-Neuronen (SGN) unerlässlich. Oxidativer Stress verursacht neuronalen Zelltod und für die Pathologie Taubheit Bedeutung. Fumarsäureester (FAEs) sind ihre antioxidativen neuroprotektiven Eigenschaften bekannt, orale Verabreichung Dimethylfumarat (DMF) eine zugelassene Behandlung Multiple Sklerose. FAEs ein vielversprechender Kandidat Neuroprotektion SGNs.
<h3></h3> Inflammation plays an important role in Huntington's disease (HD) pathology and progression. Chronic activation of microglia the release inflammatory cytokines are key processes brain found prior to symptom onset HD. A main pathway that leads microglial is JAK/STAT signaling, which was recently shown be increased putamen HD patients. Hence, we aim study JAK/STAT-mediated To induce human cell (HMC) line HMC3, cells were treated with lipopolysaccharide (LPS) interferon (INF)γ. For...