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Frontiers in Molecular Neuroscience

Kai K Kummer, Theodora Kalpachidou, Miodrag Mitrić, Michiel Langeslag, Michaela Kress
Fabry disease is an X-chromosome linked hereditary disease that is caused by loss of function mutations in the α-galactosidase A (α-Gal A) gene, resulting in defective glycolipid degradation and subsequent accumulation of globotriaosylceramide (Gb3) in different tissues, including vascular endothelial cells and neurons in the peripheral and central nervous system. We recently reported a differential gene expression profile of α-Gal A(-/0) mouse dorsal root ganglia, an established animal model of Fabry disease, thereby providing new gene targets that might underlie the neuropathic pain related symptoms...
2018: Frontiers in Molecular Neuroscience
Yu Zhang, Bing Xu, Qi Chen, Yong Yan, Jiulin Du, Xufei Du
During development, immature blood vessel networks remodel to form a simplified and efficient vasculature to meet the demand for oxygen and nutrients, and this remodeling process is mainly achieved via the pruning of existing vessels. It has already known that the migration of vascular endothelial cells (ECs) is one of the mechanisms underlying vessel pruning. However, the role of EC apoptosis in vessel pruning remains under debate, especially in the brain. Here, we reported that EC apoptosis makes a significant contribution to vessel pruning in the brain of larval zebrafish...
2018: Frontiers in Molecular Neuroscience
Chunmei Wang, Qinqin Wang, Bingyuan Ji, Yanyou Pan, Chao Xu, Baohua Cheng, Bo Bai, Jing Chen
Orexins, also known as hypocretins, are two neuropeptides secreted from orexin-containing neurons, mainly in the lateral hypothalamus (LH). Orexins orchestrate their effects by binding and activating two G-protein-coupled receptors (GPCRs), orexin receptor type 1 (OX1R) and type 2 (OX2R). Orexin/receptor pathways play vital regulatory roles in many physiological processes, especially feeding behavior, sleep-wake rhythm, reward and addiction and energy balance. Furthermore several reports showed that orexin/receptor pathways are involved in pathological processes of neurological diseases such as narcolepsy, depression, ischemic stroke, drug addiction and Alzheimer's disease (AD)...
2018: Frontiers in Molecular Neuroscience
Michelle Lindström, Beidong Liu
Fused in sarcoma (FUS) is a multifunctional DNA/RNA-binding protein predominantly localized in the cell nucleus. However, FUS has been shown to accumulate and form aggregates in the cytoplasm when mislocalized there due to mutations. These FUS protein aggregates are known as pathological hallmarks in a subset of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) cases. In this review, we discussed recent research developments on elucidating the molecular mechanisms behind FUS protein aggregation and toxicity...
2018: Frontiers in Molecular Neuroscience
Wenjuan Zhang, Sun Myoung Kim, Wenwen Wang, Cuiyuan Cai, Yong Feng, Weijia Kong, Xi Lin
Sensorineural hearing loss (SNHL) affects millions of people. Genetic mutations play a large and direct role in both congenital and late-onset cases of SNHL (e.g., age-dependent hearing loss, ADHL). Although hearing aids can help moderate to severe hearing loss the only effective treatment for deaf patients is the cochlear implant (CI). Gene- and cell-based therapies potentially may preserve or restore hearing with more natural sound perception, since their theoretical frequency resolution power is much higher than that of cochlear implants...
2018: Frontiers in Molecular Neuroscience
Timea Aczél, József Kun, Éva Szőke, Tibor Rauch, Sini Junttila, Attila Gyenesei, Kata Bölcskei, Zsuzsanna Helyes
Orofacial pain and headache disorders are among the most debilitating pain conditions. While the pathophysiological basis of these disorders may be diverse, it is generally accepted that a common mechanism behind the arising pain is the sensitization of extra- and intracranial trigeminal primary afferents. In the present study we investigated gene expression changes in the trigeminal ganglia (TRG), trigeminal nucleus caudalis (TNC) and peripheral blood mononuclear cells (PBMC) evoked by Complete Freund's Adjuvant (CFA)-induced orofacial inflammation in rats, as a model of trigeminal sensitization...
2018: Frontiers in Molecular Neuroscience
Alex Plata, Albina Lebedeva, Pavel Denisov, Olga Nosova, Tatiana Y Postnikova, Alexey Pimashkin, Alexey Brazhe, Aleksey V Zaitsev, Dmitri A Rusakov, Alexey Semyanov
Epilepsy is a group of neurological disorders commonly associated with the neuronal malfunction leading to generation of seizures. Recent reports point to a possible contribution of astrocytes into this pathology. We used the lithium-pilocarpine model of status epilepticus (SE) in rats to monitor changes in astrocytes. Experiments were performed in acute hippocampal slices 2-4 weeks after SE induction. Nissl staining revealed significant neurodegeneration in the pyramidal cell layers of hippocampal CA1, CA3 areas, and the hilus, but not in the granular cell layer of the dentate gyrus...
2018: Frontiers in Molecular Neuroscience
David M Bannerman, Thilo Borchardt, Vidar Jensen, Andrey Rozov, Nadia N Haj-Yasein, Nail Burnashev, Daniel Zamanillo, Thorsten Bus, Isabel Grube, Giselind Adelmann, J Nicholas P Rawlins, Rolf Sprengel
The GluA1 subunit of the L-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) plays a crucial, but highly selective, role in cognitive function. Here we analyzed AMPAR expression, AMPAR distribution and spatial learning in mice ( Gria1R/R ), expressing the "trafficking compromised" GluA1(Q600R) point mutation. Our analysis revealed somatic accumulation and reduction of GluA1(Q600R) and GluA2, but only slightly reduced CA1 synaptic localization in hippocampi of adult Gria1R/R mice...
2018: Frontiers in Molecular Neuroscience
Rafael Falcón-Moya, Talvinder S Sihra, Antonio Rodríguez-Moreno
Kainate (KA) is a potent neurotoxin that has been widely used experimentally to induce acute brain seizures and, after repetitive treatments, as a chronic model of temporal lobe epilepsy (TLE), with similar features to those observed in human patients with TLE. However, whether KA activates KA receptors (KARs) as an agonist to mediate the induction of acute seizures and/or the chronic phase of epilepsy, or whether epileptogenic effects of the neurotoxin are indirect and/or mediated by other types of receptors, has yet to be satisfactorily elucidated...
2018: Frontiers in Molecular Neuroscience
Michelle E Watts, Roger Pocock, Charles Claudianos
Dynamic metabolic changes occurring in neurons are critically important in directing brain plasticity and cognitive function. In other tissue types, disruptions to metabolism and the resultant changes in cellular oxidative state, such as increased reactive oxygen species (ROS) or induction of hypoxia, are associated with cellular stress. In the brain however, where drastic metabolic shifts occur to support physiological processes, subsequent changes to cellular oxidative state and induction of transcriptional sensors of oxidative stress likely play a significant role in regulating physiological neuronal function...
2018: Frontiers in Molecular Neuroscience
Andreas Aufschnaiter, Verena Kohler, Corvin Walter, Sergi Tosal-Castano, Lukas Habernig, Heimo Wolinski, Walter Keller, F-Nora Vögtle, Sabrina Büttner
Mitochondrial dysfunction is a prominent trait of cellular decline during aging and intimately linked to neuronal degeneration during Parkinson's disease (PD). Various proteins associated with PD have been shown to differentially impact mitochondrial dynamics, quality control and function, including the leucine-rich repeat kinase 2 (LRRK2). Here, we demonstrate that high levels of the enzymatic core of human LRRK2, harboring GTPase as well as kinase activity, decreases mitochondrial mass via an impairment of mitochondrial biogenesis in aging yeast...
2018: Frontiers in Molecular Neuroscience
Carolina Fabelo, Jennifer Hernandez, Rachel Chang, Sakara Seng, Natalia Alicea, Sharon Tian, Kristie Conde, Edward J Wagner
We tested the hypotheses that steroidogenic factor (SF)-1 neurons in the hypothalamic ventromedial nucleus (VMN) provide sexually disparate, endocannabinoid (EC)- and diet-sensitive glutamatergic input onto proopiomelanocortin (POMC) neurons. Electrophysiological recordings were performed in hypothalamic slices from intact and castrated guinea pigs, along with in vitro optogenetic experiments in intact male as well as cycling and ovariectomized female NR5A1-Cre mice. In slices from castrated male and female guinea pigs, depolarized-induced suppression of excitation (DSE) time-dependently reduced the amplitude of evoked excitatory postsynaptic currents (eEPSCs) in POMC neurons generated by electrically stimulating the dorsomedial VMN...
2018: Frontiers in Molecular Neuroscience
Elli-Anna Balta, Marie-Theres Wittmann, Matthias Jung, Elisabeth Sock, Benjamin Martin Haeberle, Birgit Heim, Felix von Zweydorf, Jana Heppt, Julia von Wittgenstein, Christian Johannes Gloeckner, Dieter Chichung Lie
SOX11 is a key Transcription Factor (TF) in the regulation of embryonic and adult neurogenesis, whose mutation has recently been linked to an intellectual disability syndrome in humans. SOX11's transient activity during neurogenesis is critical to ensure the precise execution of the neurogenic program. Here, we report that SOX11 displays differential subcellular localizations during the course of neurogenesis. Western-Blot analysis of embryonic mouse brain lysates indicated that SOX11 is post-translationally modified by phosphorylation...
2018: Frontiers in Molecular Neuroscience
Silke Herzer, Cassidy Hagan, Johanna von Gerichten, Vanessa Dieterle, Bogdan Munteanu, Roger Sandhoff, Carsten Hopf, Viola Nordström
Alzheimer's disease (AD) is characterized by progressive neurodegeneration and a concomitant loss of synapses and cognitive abilities. Recently, we have proposed that an alteration of neuronal membrane lipid microdomains increases neuronal resistance toward amyloid-β stress in cultured neurons and protects from neurodegeneration in a mouse model of AD. Lipid microdomains are highly enriched in a specific subclass of glycosphingolipids, termed gangliosides. The enzyme glucosylceramide synthase (GCS) catalyzes the rate-limiting step in the biosynthesis of these gangliosides...
2018: Frontiers in Molecular Neuroscience
Srimal Samaranayake, Xiufeng Song, Sergey A Vishnivetskiy, Jeannie Chen, Eugenia V Gurevich, Vsevolod V Gurevich
We determined the effects of different expression levels of arrestin-1-3A mutant with enhanced binding to light-activated rhodopsin that is independent of phosphorylation. To this end, transgenic mice that express mutant rhodopsin with zero, one, or two phosphorylation sites, instead of six in the WT mouse rhodopsin, and normal complement of WT arrestin-1, were bred with mice expressing enhanced phosphorylation-independent arrestin-1-3A mutant. The resulting lines were characterized by retinal histology (thickness of the outer nuclear layer, reflecting the number of rod photoreceptors, and the length of the outer segments, which reflects rod health), as well as single- and double-flash ERG to determine the functionality of rods and the rate of photoresponse recovery...
2018: Frontiers in Molecular Neuroscience
Christopher Heise, Jonathan M Preuss, Jan C Schroeder, Chiara R Battaglia, Jonas Kolibius, Rebecca Schmid, Michael R Kreutz, Martien J H Kas, J Peter H Burbach, Tobias M Boeckers
Autism spectrum disorder (ASD) refers to a large set of neurodevelopmental disorders, which have in common both repetitive behavior and abnormalities in social interactions and communication. Interestingly, most forms of ASD have a strong genetic contribution. However, the molecular underpinnings of this disorder remain elusive. The SHANK3 gene (and to a lesser degree SHANK2 ) which encode for the postsynaptic density (PSD) proteins SHANK3/SHANK2 and the CONTACTIN 4 gene which encodes for the neuronal glycoprotein CONTACTIN4 (CNTN4) exhibit mutated variants which are associated with ASD...
2018: Frontiers in Molecular Neuroscience
Yuting Li, Yuhua Sun, Min Cai, Huanhuan Zhang, Nannan Gao, Huiwei Huang, Shusen Cui, Dengbing Yao
Wallerian degeneration (WD) is associated with changes in the expression levels of a large number of genes. However, the effects of these up- or down-regulated genes are poorly understood. We have reported some key factors that are differentially regulated during WD in our previous research. Here, we explored the roles of Fas ligand gene (Faslg) in WD after rat sciatic nerve injury. The data showed that Faslg was up-regulated in injured nerves. Expression changed of Faslg in Schwann cells (SCs) resulted in alterations in the release of related factors...
2018: Frontiers in Molecular Neuroscience
Seungjoon Lee, Eunee Lee, Ryunhee Kim, Jihye Kim, Suho Lee, Haram Park, Esther Yang, Hyun Kim, Eunjoon Kim
Shank2 is an abundant postsynaptic scaffolding protein implicated in neurodevelopmental and psychiatric disorders, including autism spectrum disorders (ASD). Deletion of Shank2 in mice has been shown to induce social deficits, repetitive behaviors, and hyperactivity, but the identity of the cell types that contribute to these phenotypes has remained unclear. Here, we report a conditional mouse line with a Shank2 deletion restricted to parvalbumin (PV)-positive neurons ( Pv-Cre;Shank2 fl/fl mice). These mice display moderate hyperactivity in both novel and familiar environments and enhanced self-grooming in novel, but not familiar, environments...
2018: Frontiers in Molecular Neuroscience
Meera E Modi, Julie M Brooks, Edward R Guilmette, Mercedes Beyna, Radka Graf, Dominik Reim, Michael J Schmeisser, Tobias M Boeckers, Patricio O'Donnell, Derek L Buhl
Mutations in the SHANK family of genes have been consistently identified in genetic and genomic screens of autism spectrum disorder (ASD). The functional overlap of SHANK with several other ASD-associated genes suggests synaptic dysfunction as a convergent mechanism of pathophysiology in ASD. Although many ASD-related mutations result in alterations to synaptic function, the nature of those dysfunctions and the consequential behavioral manifestations are highly variable when expressed in genetic mouse models...
2018: Frontiers in Molecular Neuroscience
Kay-Marie J Lamar, Gemma L Carvill
The chromodomain helicase DNA-binding (CHD) family of proteins are ATP-dependent chromatin remodelers that contribute to the reorganization of chromatin structure and deposition of histone variants necessary to regulate gene expression. CHD proteins play an important role in neurodevelopment, as pathogenic variants in CHD1 , CHD2 , CHD4 , CHD7 and CHD8 have been associated with a range of neurological phenotypes, including autism spectrum disorder (ASD), intellectual disability (ID) and epilepsy. Pathogenic variants in CHD2 are associated with developmental epileptic encephalopathy (DEE) in humans, however little is known about how these variants contribute to this disorder...
2018: Frontiers in Molecular Neuroscience
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