Search Results - "Hensel, Niko"
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The Need for SMN-Independent Treatments of Spinal Muscular Atrophy (SMA) to Complement SMN-Enhancing Drugs
Published in Frontiers in neurology (03-02-2020)“…Spinal Muscular Atrophy (SMA) is monogenic motoneuron disease caused by low levels of the Survival of Motoneuron protein (SMN). Recently, two different drugs…”
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ERK and ROCK functionally interact in a signaling network that is compensationally upregulated in Spinal Muscular Atrophy
Published in Neurobiology of disease (01-12-2017)“…Spinal Muscular Atrophy (SMA) is a motoneuron disease caused by low levels of functional survival of motoneuron protein (SMN). Molecular disease mechanisms…”
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Fibroblast growth factor 23 signaling in hippocampal cells: impact on neuronal morphology and synaptic density
Published in Journal of neurochemistry (01-06-2016)“…Endocrine fibroblast growth factor 23 (FGF23) is predominantly secreted by osteocytes and facilitates renal phosphate excretion. However, FGF23 is also present…”
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The spinal muscular atrophy disease protein SMN is linked to the rho-kinase pathway via profilin
Published in Human molecular genetics (15-12-2011)“…Spinal muscular atrophy (SMA), a frequent neurodegenerative disease, is caused by reduced levels of functional survival of motoneuron (SMN) protein. SMN is…”
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Suppression of the necroptotic cell death pathways improves survival in Smn2B/− mice
Published in Frontiers in cellular neuroscience (03-08-2022)“…Spinal muscular atrophy (SMA) is a monogenic neuromuscular disease caused by low levels of the Survival Motor Neuron (SMN) protein. Motor neuron degeneration…”
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Therapeutic potential of mesenchymal stromal cells and MSC conditioned medium in Amyotrophic Lateral Sclerosis (ALS)--in vitro evidence from primary motor neuron cultures, NSC-34 cells, astrocytes and microglia
Published in PloS one (12-09-2013)“…Administration of mesenchymal stromal cells (MSC) improves functional outcome in the SOD1G93A mouse model of the degenerative motor neuron disorder amyotrophic…”
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The Proteome and Secretome of Cortical Brain Cells Infected With Herpes Simplex Virus
Published in Frontiers in neurology (27-08-2020)“…Infections of the brain with herpes simplex virus type 1 (HSV-1) cause life-threatening Herpes simplex encephalitis (HSE) characterized by viral replication in…”
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Analysis of the fibroblast growth factor system reveals alterations in a mouse model of spinal muscular atrophy
Published in PloS one (13-02-2012)“…The monogenetic disease Spinal Muscular Atrophy (SMA) is characterized by a progressive loss of motoneurons leading to muscle weakness and atrophy due to…”
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Bilateral crosstalk of rho- and extracellular-signal-regulated-kinase (ERK) pathways is confined to an unidirectional mode in spinal muscular atrophy (SMA)
Published in Cellular signalling (01-03-2014)“…Rho-kinase (ROCK) as well as extracellular signal regulated kinase (ERK) control actin cytoskeletal organization thereby regulating dynamic changes of cellular…”
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HSV-1 triggers paracrine fibroblast growth factor response from cortical brain cells via immediate-early protein ICP0
Published in Journal of neuroinflammation (02-12-2019)“…Herpes simplex virus-1 (HSV-1) infections of the central nervous system (CNS) can result in HSV-1 encephalitis (HSE) which is characterized by severe brain…”
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Investigations of Microtubule-associated Protein 2 Gene Expression in Spinal Muscular Atrophy
Published in The journal of pediatric research (01-06-2019)“…Aim: Spinal muscular atrophy (SMA) is a devastating genetic disease in childhood andff is caused by the absence of functional survival motor neuron (SMN)…”
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The Actin Cytoskeleton in SMA and ALS: How Does It Contribute to Motoneuron Degeneration?
Published in The Neuroscientist (01-02-2018)“…Amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA) are neurodegenerative diseases with overlapping clinical phenotypes based on impaired…”
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The phospho-landscape of the survival of motoneuron protein (SMN) protein: relevance for spinal muscular atrophy (SMA)
Published in Cellular and molecular life sciences : CMLS (01-09-2022)“…Spinal muscular atrophy (SMA) is caused by low levels of the survival of motoneuron (SMN) Protein leading to preferential degeneration of lower motoneurons in…”
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Resolution of pathogenic R-loops rescues motor neuron degeneration in spinal muscular atrophy
Published in Brain (London, England : 1878) (01-01-2020)“…This scientific commentary refers to ‘ZPR1 prevents R-loop accumulation, upregulates SMN2 expression and rescues spinal muscular atrophy’, by Kannan et al…”
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The spinal muscular atrophy gene product regulates actin dynamics
Published in The FASEB journal (30-09-2024)“…Spinal Muscular Atrophy (SMA) is a neuromuscular disease caused by low levels of the Survival of Motoneuron (SMN) protein. SMN interacts with and regulates the…”
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Profilin2a‐phosphorylation as a regulatory mechanism for actin dynamics
Published in The FASEB journal (01-02-2020)“…Profilin is a major regulator of actin dynamics in multiple specific processes localized in different cellular compartments. This specificity is not only…”
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Microtubule-associated protein 1B dysregulates microtubule dynamics and neuronal mitochondrial transport in spinal muscular atrophy
Published in Human molecular genetics (25-02-2021)“…Spinal muscular atrophy (SMA) is a devastating childhood disease primarily affecting lower motoneurons in the spinal cord. SMA is caused by the loss of…”
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Altered calcium dynamics and glutamate receptor properties in iPSC-derived motor neurons from ALS patients with C9orf72, FUS, SOD1 or TDP43 mutations
Published in Human molecular genetics (01-09-2019)“…Abstract The fatal neurodegenerative disease amyotrophic lateral sclerosis (ALS) is characterized by a profound loss of motor neurons (MNs). Until now only…”
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Chatting with the neighbors: crosstalk between Rho-kinase (ROCK) and other signaling pathways for treatment of neurological disorders
Published in Frontiers in neuroscience (02-06-2015)“…ROCK inhibition has been largely applied as a strategy to treat neurodegenerative diseases (NDDs) and promising results have been obtained in the recent years…”
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Central and peripheral delivered AAV9-SMN are both efficient but target different pathomechanisms in a mouse model of spinal muscular atrophy
Published in Gene therapy (01-09-2022)“…Spinal muscular atrophy (SMA) is a neuromuscular disease caused by loss of the SMN1 gene and low SMN protein levels. Although lower motor neurons are a primary…”
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