412 results match your criteria: "Institute of Clinical Neurobiology[Affiliation]"
Cell Rep
December 2024
Institute of Biochemistry, Christian-Albrechts-University Kiel, Olshausenstrasse 40, 24118 Kiel, Germany. Electronic address:
Genetic variants in TMEM106B, coding for a transmembrane protein of unknown function, have been identified as critical genetic modulators in various neurodegenerative diseases with a strong effect in patients with frontotemporal degeneration. The luminal domain of TMEM106B can form amyloid-like fibrils upon proteolysis. Whether this luminal domain is generated under physiological conditions and which protease(s) are involved in shedding remain unclear.
View Article and Find Full Text PDFJ Neural Transm (Vienna)
October 2024
Institute of Clinical Neurobiology, Alberichgasse 5/13, Vienna, A-1150, Austria.
Amyotrophic lateral sclerosis (ALS) is a fatal multi-system neurodegenerative disorder with no effective treatment or cure. Although primarily characterized by motor degeneration, cognitive dysfunction is an important non-motor symptom that has a negative impact on patient and caregiver burden. Mild cognitive deficits are present in a subgroup of non-demented patients with ALS, often preceding motor symptoms.
View Article and Find Full Text PDFJ Neural Transm (Vienna)
October 2024
Institute of Clinical Neurobiology, Alberichgasse 5/13, 1150, Vienna, Austria.
Patients with Parkinson disease (PD) frequently experience several behavioral symptoms, such as anxiety, apathy, irritability, agitation, impulsive control and obsessive-compulsive or REM sleep behavior disorders, which can cause severe psychosocial problems and impair quality of life. Occurring in 30-70% of PD patients, these symptoms can manifest at early stages of the disease, sometimes even before the appearance of classic motor symptoms, while others can develop later. Behavioral changes in PD show distinct patterns of brain atrophy, dopaminergic and serotonergic deterioration, altered neuronal connectivity in frontostriatal, corticolimbic, default mode and other networks due to a cascade linking molecular pathologies and deficits in multiple behavior domains.
View Article and Find Full Text PDFFront Immunol
October 2024
Department of Neurology, University Hospital Würzburg, Würzburg, Germany.
Introduction: Autoimmune nodopathy (AN) is a new entity in the field of peripheral neuropathies and is defined by the presence of auto-antibodies against structures of the node of Ranvier combined with specific clinico-pathophysiological features and therapy response in affected patients. The target-specific antibodies do not only serve as diagnostic biomarkers but also for treatment evaluation during follow-up.
Case Report: We report a 66-year-old female patient with various autoimmune diseases, including a history of membranous glomerulonephritis which presented with acute-onset, sensorimotor tetraparesis, cranial nerve involvement, and mild respiratory insufficiency.
J Neural Transm (Vienna)
October 2024
Institute of Clinical Neurobiology, Alberichgasse 5/13, Vienna, A-1150, Austria.
Nat Commun
October 2024
Institute of Clinical Neurobiology, University Hospital Würzburg, Würzburg, Germany.
Increasing evidence suggests an essential function for autophagy in unconventional protein secretion (UPS). However, despite its relevance for the secretion of aggregate-prone proteins, the mechanisms of secretory autophagy in neurons have remained elusive. Here we show that the lower motoneuron disease-associated guanine exchange factor Plekhg5 drives the UPS of Sod1.
View Article and Find Full Text PDFMov Disord
December 2024
Banner Sun Health Research Institute, Sun City, Arizona, USA.
Cells
September 2024
Institute of Pathology, Department of Neuropathology, University of Wuerzburg, 97080 Wuerzburg, Germany.
Postencephalitic parkinsonism (PEP) is suggested to show a virus-induced pathology, which is different from classical idiopathic Parkinson's disease (PD) as there is no α-synuclein/Lewy body pathology. However, PEP shows a typical clinical representation of motor disturbances. In addition, compared to PD, there is no iron-induced pathology.
View Article and Find Full Text PDFCells
September 2024
Institute of Clinical Neurobiology, University Hospital Wuerzburg, Versbacher Str. 5, 97078 Würzburg, Germany.
ER-phagy is a specialized form of autophagy, defined by the lysosomal degradation of ER subdomains. ER-phagy has been implicated in relieving the ER from misfolded proteins during ER stress upon activation of the unfolded protein response (UPR). Here, we identified an essential role for the ER chaperone calnexin in regulating ER-phagy and the UPR in neurons.
View Article and Find Full Text PDFNat Rev Neurosci
November 2024
Defense Circuits Lab, Institute of Clinical Neurobiology, University Hospital Würzburg, Würzburg, Germany.
Front Mol Neurosci
August 2024
Institute of Clinical Neurobiology, University Hospital Würzburg, Würzburg, Germany.
Spinal muscular atrophy (SMA) is a neuromuscular disorder caused by mutations or deletions in the survival motoneuron 1 () gene, resulting in deficiency of the SMN protein that is essential for motoneuron function. Smn depletion in mice disturbs axonal RNA transport and translation, thereby contributing to axon growth impairment, muscle denervation, and motoneuron degeneration. However, the mechanisms whereby Smn loss causes axonal defects remain unclear.
View Article and Find Full Text PDFJ Neural Transm (Vienna)
September 2024
Institute of Clinical Neurobiology, Alberichgasse 5/13, Vienna, A-1150, Austria.
Dementia with Lewy bodies (DLB), the second most common primary degenerative neurocognitive disorder after Alzheimer disease, is frequently preceded by REM sleep behavior disorders (RBD) and other behavioral symptoms, like anxiety, irritability, agitation or apathy, as well as visual hallucinations and delusions, most of which occurring in 40-60% of DLB patients. Other frequent behavioral symptoms like attention deficits contribute to cognitive impairment, while attention-deficit/hyperactivity disorder (ADHD) is a risk factor for DLB. Behavioral problems in DLB are more frequent, more severe and appear earlier than in other neurodegenerative diseases and, together with other neuropsychiatric symptoms, contribute to impairment of quality of life of the patients, but their pathophysiology is poorly understood.
View Article and Find Full Text PDFJ Neural Transm (Vienna)
September 2024
Institute of Clinical Neurobiology, Alberichgasse 5/13, Vienna, A-1150, Austria.
Neurol Neuroimmunol Neuroinflamm
November 2024
From the Division of Neuropathology and Neurochemistry (M.W., E.G., R.H.), Department of Neurology, Medical University of Vienna; Department of Neurology (M.W.), Johannes Kepler University Linz, Kepler University Hospital, Linz, Austria; Section of Translational Neuroimmunology (J.W., D.B., A.G., C.G.), Department of Neurology, Jena University Hospital, Germany; Comprehensive Center for Clinical Neurosciences and Mental Health (E.G., R.H.), Medical University of Vienna, Austria; Institute of Clinical Neurobiology (V.R., C.V.), University Hospital, Julius-Maximilians-University of Würzburg, Germany; Department of Neuroimmunology (J.B.), Center for Brain Research; and Center for Medical Physics and Biomedical Engineering (A.S.S., P.J.), Medical University of Vienna, Austria.
Biomedicines
August 2024
Institute of Clinical Neurobiology, University Hospital Wuerzburg, 97078 Wuerzburg, Germany.
Neurotrophins, particularly brain-derived neurotrophic factor (BDNF), act as key regulators of neuronal development, survival, and plasticity. BDNF is necessary for neuronal and functional maintenance in the striatum and the substantia nigra, both structures involved in the pathogenesis of Parkinson's Disease (PD). Depletion of BDNF leads to striatal degeneration and defects in the dendritic arborization of striatal neurons.
View Article and Find Full Text PDFNat Commun
August 2024
Institute of Clinical Neurobiology, University Hospital Wuerzburg, Wuerzburg, Germany.
Motoneurons critically depend on precise spatial and temporal control of translation for axon growth and the establishment and maintenance of neuromuscular connections. While defects in local translation have been implicated in the pathogenesis of motoneuron disorders, little is known about the mechanisms regulating axonal protein synthesis. Here, we report that motoneurons derived from Hnrnpr knockout mice show reduced axon growth accompanied by lowered synthesis of cytoskeletal and synaptic components in axons.
View Article and Find Full Text PDFNPJ Parkinsons Dis
August 2024
Department of Neurology, University Hospital of Würzburg, Josef-Schneider-Straße 11, 97080, Würzburg, Germany.
Deep brain stimulation (DBS) has emerged as a revolutionary technique for accessing and modulating brain circuits. DBS is used to treat dysfunctional neuronal circuits in neurological and psychiatric disorders. Despite over two decades of clinical application, the fundamental mechanisms underlying DBS are still not well understood.
View Article and Find Full Text PDFCancer Gene Ther
September 2024
Department of Neurology, University Hospital Würzburg, Würzburg, Germany.
Trk (NTRK) receptor and NTRK gene fusions are oncogenic drivers of a wide variety of tumors. Although Trk receptors are typically activated at the cell surface, signaling of constitutive active Trk and diverse intracellular NTRK fusion oncogenes is barely investigated. Here, we show that a high intracellular abundance is sufficient for neurotrophin-independent, constitutive activation of TrkB kinase domains.
View Article and Find Full Text PDFJ Parkinsons Dis
July 2024
Director of the Institute of Clinical Neurobiology, Vienna, Austria.
Int J Mol Sci
July 2024
Institute of Clinical Neurobiology, Alberichgasse 5/13, 1150 Vienna, Austria.
While cognitive impairment, which was previously considered a red flag against the clinical diagnosis of multiple system atrophy (MSA), is a common symptom of this rare neurodegenerative disorder, behavioral disorders are reported in 30 to 70% of MSA patients. They include anxiety, apathy, impaired attention, compulsive and REM sleep behavior disorders (RBD), and these conditions, like depression, are early and pervasive features in MSA, which may contribute to disease progression. Despite changing concepts of behavioral changes in this synucleinopathy, the underlying pathophysiological and biochemical mechanisms are poorly understood.
View Article and Find Full Text PDFJ Neural Transm (Vienna)
September 2024
Institute of Clinical Neurobiology, Alberichgasse 5/13, Vienna, A-1150, Austria.
Huntington disease (HD), a devastating autosomal-dominant neurodegenerative disease caused by an expanded CAG trinucleotide repeat, is clinically characterized by a triad of symptoms including involuntary motions, behavior problems and cognitive deficits. Behavioral symptoms with anxiety, irritability, obsessive-compulsive behaviors, apathy and other neuropsychiatric symptoms, occurring in over 50% of HD patients are important features of this disease and contribute to impairment of quality of life, but their pathophysiology is poorly understood. Behavior problems, more frequent than depression, can be manifest before obvious motor symptoms and occur across all HD stages, usually correlated with duration of illness.
View Article and Find Full Text PDFJ Neural Transm (Vienna)
August 2024
Institute of Clinical Neurobiology, Alberichgasse 5/13, Vienna, A-1150, Austria.
Depression and anxiety are the most frequent neuropsychiatric symptoms of multiple sclerosis (MS), an autoimmune-mediated demyelinating neurodegenerative disease. Their prevalence is 25-65% and 20-54%, respectively, often associated with chronic fatigue and cognitive impairment, but usually not correlated with motor and other deficits, suggesting different pathophysiological mechanisms. Both disorders often arise before MS diagnosis, lead to faster disability and impair the quality of life.
View Article and Find Full Text PDFElife
June 2024
Institute of Pharmacology and Toxicology, University of Würzburg, Würzburg, Germany.
Vitamin B6 deficiency has been linked to cognitive impairment in human brain disorders for decades. Still, the molecular mechanisms linking vitamin B6 to these pathologies remain poorly understood, and whether vitamin B6 supplementation improves cognition is unclear as well. Pyridoxal 5'-phosphate phosphatase (PDXP), an enzyme that controls levels of pyridoxal 5'-phosphate (PLP), the co-enzymatically active form of vitamin B6, may represent an alternative therapeutic entry point into vitamin B6-associated pathologies.
View Article and Find Full Text PDFJ Neural Transm (Vienna)
August 2024
Institute of Clinical Neurobiology, Alberichgasse 5/13, Vienna, A-1150, Austria.
Sci Rep
May 2024
JPF Analytics, Jnana Prabodhini Foundation, Murrieta, CA, USA.
Across the world, the officially reported number of COVID-19 deaths is likely an undercount. Establishing true mortality is key to improving data transparency and strengthening public health systems to tackle future disease outbreaks. In this study, we estimated excess deaths during the COVID-19 pandemic in the Pune region of India.
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