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Advances in Neurobiology

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https://www.readbyqxmd.com/read/30334227/not-cure-but-heal-music-and-medicine
#1
Paulo E Andrade, Joydeep Bhattacharya
Do you know that our soul is composed of harmony? Leonardo Da Vinci Despite evidence for music-specific mechanisms at the level of pitch-pattern representations, the most fascinating aspect of music is its transmodality. Recent psychological and neuroscientific evidence suggest that music is unique in the coupling of perception, cognition, action and emotion. This potentially explains why music has been since time immemorial almost inextricably linked to healing processes and should continue to be.
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/30334226/using-systems-biology-and-mathematical-modeling-approaches-in-the-discovery-of-therapeutic-targets-for-spinal-muscular-atrophy
#2
Matthew E R Butchbach
Systems biology uses a combination of experimental and mathematical approaches to investigate the complex and dynamic interactions with a given system or biological process. Systems biology integrates genetics, signal transduction, biochemistry and cell biology with mathematical modeling. It can be used to identify novel pathways implicated in diseases as well as to understand the mechanisms by which a specific gene is regulated. This review describes the development of mathematical models for the regulation of an endogenous modifier gene, SMN2, in spinal muscular atrophy-an early-onset motor neuron disease that is a leading genetic cause of infant mortality worldwide-by cAMP signaling...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/30334225/large-de-novo-microdeletion-in-epilepsy-with-intellectual-and-developmental-disabilities-with-a-systems-biology-analysis
#3
Kai Gao, Yujia Zhang, Ling Zhang, Weijing Kong, Han Xie, Jingmin Wang, Ye Wu, Xiru Wu, Xiaoyan Liu, Yuehua Zhang, Feng Zhang, Albert Cheung-Hoi Yu, Yuwu Jiang
Epilepsy is one of the most common complex neurological diseases. It is frequently associated with intellectual and developmental disabilities (ID/DD). In recent years, copy number variation (CNV), especially microdeletion, was proven to be a potential key factor of genetic epilepsy. In this paper, the authors tested the hypothesis that the large de novo rare CNV is an important cause of epilepsy with ID/DD. We performed a custom array comparative genomic hybridization (aCGH) to detect the CNVs of 96 Chinese epileptic patients with ID/DD...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/30334224/synaptic-plasticity-and-synchrony-in-the-anterior-cingulate-cortex-circuitry-a-neural-network-approach-to-causality-of-chronic-visceral-pain-and-associated-cognitive-deficits
#4
Ying Li
Human brain imaging studies have demonstrated the importance of cortical neuronal networks in the perception of pain in patients with functional bowel disease such as irritable bowel syndrome (IBS).Studies have identified an enhanced response in the anterior cingulate cortex (ACC) to colorectal distension in viscerally hypersensitive (VH) rats. Electrophysiological recordings show long-lasting potentiation of local field potential (LFP) in the medial thalamus (MT)-ACC synapses in VH rats. Theta burst stimulation in the MT reliably induced long-term potentiation (LTP) in the MT-ACC pathway in normal rats, but was occluded in the VH state...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/30334223/genome-scale-brain-metabolic-networks-as-scaffolds-for-the-systems-biology-of-neurodegenerative-diseases-mapping-metabolic-alterations
#5
Emrah Özcan, Tunahan Çakır
Systems-based investigation of diseases requires integrated analysis of cellular networks and high-throughput data of gene products. The use of genome-scale metabolic networks for such integration has led to the elucidation of cellular mechanisms for several cell types from microorganisms to plants. It has become easier and cheaper to generate high-throughput data over years in the form of transcriptome, proteome and metabolome. This has tremendously improved the quality and quantity of information extracted from such data enabling the documentation of active pathways and reactions in cell metabolism...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/30334222/mapping-molecular-datasets-back-to-the-brain-regions-they-are-extracted-from-remembering-the-native-countries-of-hypothalamic-expatriates-and-refugees
#6
Arshad M Khan, Alice H Grant, Anais Martinez, Gully A P C Burns, Brendan S Thatcher, Vishwanath T Anekonda, Benjamin W Thompson, Zachary S Roberts, Daniel H Moralejo, James E Blevins
This article focuses on approaches to link transcriptomic, proteomic, and peptidomic datasets mined from brain tissue to the original locations within the brain that they are derived from using digital atlas mapping techniques. We use, as an example, the transcriptomic, proteomic and peptidomic analyses conducted in the mammalian hypothalamus. Following a brief historical overview, we highlight studies that have mined biochemical and molecular information from the hypothalamus and then lay out a strategy for how these data can be linked spatially to the mapped locations in a canonical brain atlas where the data come from, thereby allowing researchers to integrate these data with other datasets across multiple scales...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/30334221/synaptic-excitatory-inhibitory-balance-underlying-efficient-neural-coding
#7
Shanglin Zhou, Yuguo Yu
Both theoretical and experimental evidence indicate that synaptic excitation and inhibition in the cerebral cortex are well-balanced during the resting state and sensory processing. Here, we briefly summarize the evidence for how neural circuits are adjusted to achieve this balance. Then, we discuss how such excitatory and inhibitory balance shapes stimulus representation and information propagation, two basic functions of neural coding. We also point out the benefit of adopting such a balance during neural coding...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/30334220/context-dependent-adjustments-in-executive-control-of-goal-directed-behaviour-contribution-of-frontal-brain-areas-to-conflict-induced-behavioural-adjustments-in-primates
#8
Farshad A Mansouri, Mark J Buckley
Psychophysical studies in humans indicate that the performance in various tasks is affected by contextual factors such as conflict level and error commission. It is generally believed that contextual factors influence the executive control processes and consequently modulate ongoing behaviour. Imaging studies suggest that dorsolateral prefrontal cortex and anterior cingulate cortex play crucial roles in mediating these context-dependent adjustments in executive control of behaviour. However, the underlying neuronal processes are to a great extent unknown...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/30334219/the-hippocampal-ensemble-code-for-spatial-navigation-and-episodic-memory
#9
Susumu Takahashi
In our minds we can vividly re-experience a series of past events or episodes that occurred along space and time. In 1957, a highly influential clinical investigation published by Scoville and Milner suggested that episodic memory retrieval is severely impaired after physical damage occurs to the hippocampus. In fact, loss of the hippocampus can lead to a profound disturbance to spatial navigation. Within the hippocampus reside place cells: excitatory pyramidal cells that maximally fire at a particular location...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/30334218/neural-circuits-mediating-fear-learning-and-extinction
#10
Roger Marek, Pankaj Sah
The activity of neural circuits that underpin particular behaviours are one of the most interesting questions in neurobiology today. This understanding will not only lead to a detailed understanding of learning and memory formation, but also provides a platform for the development of novel therapeutic approaches to a range of neurological disorders that afflict humans. Among the different behavioural paradigms, Pavlovian fear conditioning and its extinction are two of the most extensively used to study acquisition, consolidation and retrieval of fear-related memories...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/30334217/neuronal-bases-of-systemic-organization-of-behavior
#11
Yuri I Alexandrov, Alexey A Sozinov, Olga E Svarnik, Alexander G Gorkin, Evgeniya A Kuzina, Vladimir V Gavrilov
Despite the years of studies in the field of systems neuroscience, functions of neural circuits and behavior-related systems are still not entirely clear. The systems description of brain activity has recently been associated with cognitive concepts, e.g. a cognitive map, reconstructed via place-cell activity analysis and the like, and a cognitive schema, modeled in consolidation research. The issue we find of importance is that a cognitive unit reconstructed in neuroscience research is mainly formulated in terms of environment...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/29916024/lost-in-translation-evidence-for-protein-synthesis-deficits-in-als-ftd-and-related-neurodegenerative-diseases
#12
REVIEW
Erik M Lehmkuhl, Daniela C Zarnescu
Cells utilize a complex network of proteins to regulate translation, involving post-transcriptional processing of RNA and assembly of the ribosomal unit. Although the complexity provides robust regulation of proteostasis, it also offers several opportunities for translational dysregulation, as has been observed in many neurodegenerative disorders. Defective mRNA localization, mRNA sequatration, inhibited ribogenesis, mutant tRNA synthetases, and translation of hexanucleotide expansions have all been associated with neurodegenerative disease...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/29916023/senataxin-a-novel-helicase-at-the-interface-of-rna-transcriptome-regulation-and-neurobiology-from-normal-function-to-pathological-roles-in-motor-neuron-disease-and-cerebellar-degeneration
#13
REVIEW
Craig L Bennett, Albert R La Spada
Senataxin (SETX) is a DNA-RNA helicase whose C-terminal region shows homology to the helicase domain of the yeast protein Sen1p. Genetic discoveries have established the importance of SETX for neural function, as recessive mutations in the SETX gene cause Ataxia with Oculomotor Apraxia type 2 (AOA2) (OMIM: 606002), which is the third most common form of recessive ataxia, after Friedreich's ataxia and Ataxia-Telangiectasia. In addition, rare, dominant SETX mutations cause a juvenile-onset form of Amyotrophic Lateral Sclerosis (ALS), known as ALS4...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/29916022/mechanisms-associated-with-tdp-43-neurotoxicity-in-als-ftld
#14
REVIEW
Marc Shenouda, Ashley B Zhang, Anna Weichert, Janice Robertson
The discovery of TDP-43 as a major disease protein in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) was first made in 2006. Prior to 2006 there were only 11 publications related to TDP-43, now there are over 2000, indicating the importance of TDP-43 to unraveling the complex molecular mechanisms that underpin the pathogenesis of ALS/FTLD. Subsequent to this discovery, TDP-43 pathology was also found in other neurodegenerative diseases, including Alzheimer's disease, the significance of which is still in the early stages of exploration...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/29916021/deregulation-of-rna-metabolism-in-microsatellite-expansion-diseases
#15
REVIEW
Chaitali Misra, Feikai Lin, Auinash Kalsotra
RNA metabolism impacts different steps of mRNA life cycle including splicing, polyadenylation, nucleo-cytoplasmic export, translation, and decay. Growing evidence indicates that defects in any of these steps lead to devastating diseases in humans. This chapter reviews the various RNA metabolic mechanisms that are disrupted in Myotonic Dystrophy-a trinucleotide repeat expansion disease-due to dysregulation of RNA-Binding Proteins. We also compare Myotonic Dystrophy to other microsatellite expansion disorders and describe how some of these mechanisms commonly exert direct versus indirect effects toward disease pathologies...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/29916020/stress-granules-and-als-a-case-of-causation-or-correlation
#16
REVIEW
Nikita Fernandes, Nichole Eshleman, J Ross Buchan
Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease characterized by cytoplasmic protein aggregates within motor neurons. These aggregates are linked to ALS pathogenesis. Recent evidence has suggested that stress granules may aid the formation of ALS protein aggregates. Here, we summarize current understanding of stress granules, focusing on assembly and clearance. We also assess the evidence linking alterations in stress granule formation and dynamics to ALS protein aggregates and disease pathology...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/29916019/rnp-assembly-defects-in-spinal-muscular-atrophy
#17
REVIEW
Phillip L Price, Dmytro Morderer, Wilfried Rossoll
Spinal muscular atrophy (SMA) is a motor neuron disease caused by mutations/deletions within the survival of motor neuron 1 (SMN1) gene that lead to a pathological reduction of SMN protein levels. SMN is part of a multiprotein complex, functioning as a molecular chaperone that facilitates the assembly of spliceosomal small nuclear ribonucleoproteins (snRNP). In addition to its role in spliceosome formation, SMN has also been found to interact with mRNA-binding proteins (mRBPs), and facilitate their assembly into mRNP transport granules...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/29916018/rna-degradation-in-neurodegenerative-disease
#18
REVIEW
Kaitlin Weskamp, Sami J Barmada
Ribonucleic acid (RNA) homeostasis is dynamically modulated in response to changing physiological conditions. Tight regulation of RNA abundance through both transcription and degradation determines the amount, timing, and location of protein translation. This balance is of particular importance in neurons, which are among the most metabolically active and morphologically complex cells in the body. As a result, any disruptions in RNA degradation can have dramatic consequences for neuronal health. In this chapter, we will first discuss mechanisms of RNA stabilization and decay...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/29916017/rna-nucleocytoplasmic-transport-defects-in-neurodegenerative-diseases
#19
REVIEW
Ashley Boehringer, Robert Bowser
In eukaryotic cells, transcription and translation are compartmentalized by the nuclear membrane, requiring an active transport of RNA from the nucleus into the cytoplasm. This is accomplished by a variety of transport complexes that contain either a member of the exportin family of proteins and translocation fueled by GTP hydrolysis or in the case of mRNA by complexes containing the export protein NXF1. Recent evidence indicates that RNA transport is altered in a number of different neurodegenerative diseases including Huntington's disease, Alzheimer's disease, frontotemporal dementia, and amyotrophic lateral sclerosis...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/29916016/rna-editing-deficiency-in-neurodegeneration
#20
REVIEW
Ileana Lorenzini, Stephen Moore, Rita Sattler
The molecular process of RNA editing allows changes in RNA transcripts that increase genomic diversity. These highly conserved RNA editing events are catalyzed by a group of enzymes known as adenosine deaminases acting on double-stranded RNA (ADARs). ADARs are necessary for normal development, they bind to over thousands of genes, impact millions of editing sites, and target critical components of the central nervous system (CNS) such as glutamate receptors, serotonin receptors, and potassium channels. Dysfunctional ADARs are known to cause alterations in CNS protein products and therefore play a role in chronic or acute neurodegenerative and psychiatric diseases as well as CNS cancer...
2018: Advances in Neurobiology
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