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https://www.readbyqxmd.com/read/28533743/neonatal-hypoxia-ischaemia-mechanisms-models-and-therapeutic-challenges
#1
REVIEW
Lancelot J Millar, Lei Shi, Anna Hoerder-Suabedissen, Zoltán Molnár
Neonatal hypoxia-ischaemia (HI) is the most common cause of death and disability in human neonates, and is often associated with persistent motor, sensory, and cognitive impairment. Improved intensive care technology has increased survival without preventing neurological disorder, increasing morbidity throughout the adult population. Early preventative or neuroprotective interventions have the potential to rescue brain development in neonates, yet only one therapeutic intervention is currently licensed for use in developed countries...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28523551/epigenetic-mechanisms-of-gene-regulation-in-amyotrophic-lateral-sclerosis
#2
Alba Jimenez-Pacheco, Jaime M Franco, Soledad Lopez, Juan Miguel Gomez-Zumaquero, Maria Magdalena Leal-Lasarte, Diana E Caballero-Hernandez, Marta Cejudo-Guillén, David Pozo
Despite being clinically described 150 years ago, the mechanisms underlying amyotrophic lateral sclerosis (ALS) pathogenesis have not yet been fully understood. Studies in both animal models of ALS and human patients reveal a plethora of alterations such as increased glutamate-mediated excitotoxicity, redox stress, increased apoptosis, defective axonal transport, protein-misfolding events, mitochondrial impairment and sustained unregulated immune responses. Regardless of being sporadic or familiar ALS, the final outcome at the cellular level is the death of upper and lower motor neurons, and once diagnosed, ALS is typically lethal within the next 5 years...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28522413/microglia-support-atf3-positive-neurons-following-hypoglossal-nerve-axotomy
#3
Tatsuhide Tanaka, Koichi Murakami, Yoshio Bando, Taichi Nomura, Ayami Isonishi, Shoko Morita-Takemura, Kouko Tatsumi, Akio Wanaka, Shigetaka Yoshida
Microglia are essential in developmental processes and maintenance of neuronal homeostasis. Experimental axotomy of motor neurons results in neurodegeneration, and microglia in motor nuclei become activated and migrate towards injured neurons. However, whether these activated microglia are protective or destructive to neurons remains controversial. In the present study, we transected the hypoglossal nerve in BALB/c mice, causing activating transcription factor 3 (ATF3) and growth associated protein 43 (GAP43) induction, and partial neuronal death...
May 15, 2017: Neurochemistry International
https://www.readbyqxmd.com/read/28520872/glucocerebrosidase-deficiency-in-dopaminergic-neurons-induces-microglial-activation-without-neurodegeneration
#4
Federico N Soria, Michel Engeln, Marta Martinez-Vicente, Christelle Glangetas, María José López-González, Sandra Dovero, Benjamin Dehay, Elisabeth Normand, Miquel Vila, Alexandre Favereaux, François Georges, Christophe Lo Bianco, Erwan Bezard, Pierre-Olivier Fernagut
Mutations in the GBA1 gene encoding the lysosomal enzyme glucocerebrosidase (GBA1) are important risk factors for Parkinson's disease (PD). In vitro, altered GBA1 activity promotes alpha-synuclein accumulation while elevated levels of alpha-synuclein compromise GBA1 function, thus supporting a pathogenic mechanism in PD. However, the mechanisms by which GBA1 deficiency is linked to increased risk of PD remains elusive, partially because of lack of aged models of GBA1 deficiency. Since knocking-out GBA1 in the entire brain induces massive neurodegeneration and early death, we generated a mouse model of GBA1 deficiency amenable to investigate the long-term consequences of compromised GBA1 function in dopaminergic neurons...
May 17, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28512398/protein-homeostasis-in-amyotrophic-lateral-sclerosis-therapeutic-opportunities
#5
REVIEW
Christopher P Webster, Emma F Smith, Pamela J Shaw, Kurt J De Vos
Protein homeostasis (proteostasis), the correct balance between production and degradation of proteins, is essential for the health and survival of cells. Proteostasis requires an intricate network of protein quality control pathways (the proteostasis network) that work to prevent protein aggregation and maintain proteome health throughout the lifespan of the cell. Collapse of proteostasis has been implicated in the etiology of a number of neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), the most common adult onset motor neuron disorder...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28507510/enhanced-axonal-extension-of-subcortical-projection-neurons-isolated-from-murine-embryonic-cortex-using-neuropilin-1
#6
Noritaka Sano, Takafumi Shimogawa, Hideya Sakaguchi, Yoshihiko Ioroi, Yoshifumi Miyawaki, Asuka Morizane, Susumu Miyamoto, Jun Takahashi
The cerebral cortical tissue of murine embryo and pluripotent stem cell (PSC)-derived neurons can survive in the brain and extend axons to the spinal cord. For efficient cell integration to the corticospinal tract (CST) after transplantation, the induction or selection of cortical motor neurons is important. However, precise information about the appropriate cell population remains unclear. To address this issue, we isolated cells expressing Neuropilin-1 (NRP1), a major axon guidance molecule receptor during the early developmental stage, from E14...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28502804/wdr79-tcab1-plays-a-conserved-role-in-the-control-of-locomotion-and-ameliorates-phenotypic-defects-in-sma-models
#7
Maria Laura Di Giorgio, Alessandro Esposito, Paolo Maccallini, Emanuela Micheli, Francesca Bavasso, Ivan Gallotta, Fiammetta Vernì, Fabian Feiguin, Stefano Cacchione, Brian D McCabe, Elia Di Schiavi, Grazia Daniela Raffa
SMN (Survival Motor Neuron) deficiency is the predominant cause of spinal muscular atrophy (SMA), a severe neurodegenerative disorder that can lead to progressive paralysis and death. Although SMN is required in every cell for proper RNA metabolism, the reason why its loss is especially critical in the motor system is still unclear. SMA genetic models have been employed to identify several modifiers that can ameliorate the deficits induced by SMN depletion. Here we focus on WDR79/TCAB1, a protein important for the biogenesis of several RNA species that has been shown to physically interact with SMN in human cells...
May 11, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28500758/mscs-ameliorates-dpn-induced-cellular-pathology-via-ca-2-i-homeostasis-and-scavenging-the-pro-inflammatory-cytokines
#8
Harish C Chandramoorthy, Ismaeel Bin-Jaliah, Hussian Karari, Prasanna Rajagopalan, Mohammed Eajaz Ahmed Shariff, Ahmed Al-Hakami, Suliman M Al-Humayad, Fawzi A Baptain, Humeda Suekit Ahmed, Hanaa Z Yassin, Mohamed A Haidara
The MSCs of various origins are known to ameliorate or modulate cell survival strategies. We investigated, whether UCB MSCs could improve the survival of the human neuronal cells and/or fibroblast assaulted with DPN sera. The results showed, the co-culture of UCB MSCs with human neuronal cells and/or fibroblasts could effectively scavenge the pro-inflammatory cytokines TNF-α, IL-1β, IFN-γ and IL - 12 and control the pro-apoptotic expression of p53/Bax. Further co-culture of UCB MSCs have shown to induce anti-inflammatory cytokines like IL-4, IL-10 and TGF-β and anti-apoptotic Bclxl/Bcl2 expression in the DPN sera stressed cells...
May 13, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28500219/mechanisms-of-enhanced-phrenic-long-term-facilitation-in-sod1-g93a-rats
#9
Nicole L Nichols, Irawan Satriotomo, Latoya L Allen, Ashley M Grebe, Gordon S Mitchell
Amyotrophic lateral sclerosis (ALS) is a degenerative motor neuron disease, causing muscle paralysis and death from respiratory failure. Effective means to preserve/restore ventilation are necessary to increase the quality and duration of life in ALS patients. At disease end-stage in a rat ALS model (SOD1(G93A)), acute intermittent hypoxia (AIH) restores phrenic nerve activity to normal levels via enhanced phrenic long-term facilitation (pLTF). Mechanisms enhancing pLTF in end-stage SOD1(G93A) rats are not known...
May 12, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28495450/rho-guanine-nucleotide-exchange-factor-rgnef-is-a-prosurvival-factor-under-stress-conditions
#10
Kevin Cheung, Cristian A Droppelmann, Adam MacLellan, Ian Cameron, Benjamin Withers, Danae Campos-Melo, Kathryn Volkening, Michael J Strong
Rho guanine nucleotide exchange factor (RGNEF) is a 190kDa RNA binding protein (RBP) that also contains a Dbl/PH domain capable of RhoA activation. Consistent with a key role in the pathogenesis of amyotrophic lateral sclerosis (ALS), RGNEF forms pathological neuronal cytoplasmic inclusions in degenerating spinal motor neurons. To further understand the role of RGNEF in the stress response, we first observed that the expression of RGNEF is upregulated in murine spinal motor neurons following distal sciatic nerve injury...
May 8, 2017: Molecular and Cellular Neurosciences
https://www.readbyqxmd.com/read/28488337/affinity-immobilization-of-vegf-on-laminin-porous-sponge-enhances-angiogenesis-in-the-ischemic-brain
#11
Mio Oshikawa, Kei Okada, Naoko Kaneko, Kazunobu Sawamoto, Itsuki Ajioka
Ischemic brain stroke is caused by blood flow interruption, leading to focal ischemia, neuron death, and motor, sensory, and/or cognitive dysfunctions. Angiogenesis, neovascularization from existing blood vessel, is essential for tissue growth and repair. Proangiogenic therapy for stroke is promising for preventing excess neuron death and improving functional recovery. Vascular endothelial growth factor (VEGF) is a critical factor for angiogenesis by promoting the proliferation, the survival, and the migration of endothelial cells...
May 10, 2017: Advanced Healthcare Materials
https://www.readbyqxmd.com/read/28487080/astrocytic-expression-of-the-rna-regulator-hur-accentuates-spinal-cord-injury-in-the-acute-phase
#12
Thaddaeus Kwan, Candace L Floyd, Jason Patel, Amanda Mohaimany-Aponte, Peter H King
We recently showed that the RNA regulator, HuR, is translocated to the cytoplasm in astrocytes in the acute phase of spinal cord injury (SCI), consistent with its activation. HuR positively modulates expression of many pro-inflammatory factors, including IL-1β, TNF-α, and MMP-12, which are present at high levels in the early phase of SCI and exacerbate tissue damage. Knockdown of HuR in astrocytes blunts expression of these factors in an in vitro stretch injury model of CNS trauma. In this report, we further investigate the impact of HuR in early SCI using a mouse model in which human HuR is transgenically expressed in astrocytes...
May 6, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28485722/how-the-discovery-of-iss-n1-led-to-the-first-medical-therapy-for-spinal-muscular-atrophy
#13
REVIEW
N N Singh, M D Howell, E J Androphy, R N Singh
Spinal muscular atrophy (SMA), a prominent genetic disease of infant mortality, is caused by low levels of survival motor neuron (SMN) protein owing to deletions or mutations of the SMN1 gene. SMN2, a nearly identical copy of SMN1 present in humans, cannot compensate for the loss of SMN1 due to predominant skipping of exon 7 during pre-mRNA splicing. With the recent FDA approval of nusinersen (Spinraza™), the potential for correction of SMN2 exon 7 splicing as a SMA therapy has been affirmed. Nusinersen is an antisense oligonucleotide that targets intronic splicing silencer N1 (ISS-N1) discovered in 2004 at the University of Massachusetts Medical School...
May 9, 2017: Gene Therapy
https://www.readbyqxmd.com/read/28482594/a-bridging-sf-alg-composite-scaffold-loaded-ngf-for-spinal-cord-injury-repair
#14
Genlong Jiao, Yongqin Pan, Cunchuang Wang, ZhaoXia Li, Zhizhong Li, Rui Guo
Neurons loss and axons degeneration after spinal cord injury (SCI) gradually give rise to result in functional motor and sensory impairment. A bridging biomaterial scaffold that allows the axons to grow through has been investigated for the repair of injured spinal cord. In this study, we introduced a silk fibroin (SF)-based neurobridge as scaffold enriched with/without nerve growth factor (NGF) that can be utilized as a therapeutic approach for spinal cord repair. NGF released from alginate (Alg) microspheres on SF scaffold (SF/Alg composites scaffolds) to the central lesion site of SCI significantly enhanced the sparing of spinal cord tissue and increased the number of surviving neurons...
July 1, 2017: Materials Science & Engineering. C, Materials for Biological Applications
https://www.readbyqxmd.com/read/28481536/discovery-of-a-small-molecule-probe-that-post-translationally-stabilizes-the-survival-motor-neuron-protein-for-the-treatment-of-spinal-muscular-atrophy
#15
Anne Rietz, Hongxia Li, Kevin M Quist, Jonathan J Cherry, Christian L Lorson, Barrington G Burnett, Nicholas L Kern, Alyssa N Calder, Melanie Fritsche, Hrvoje Lusic, Patrick J Boaler, Sungwoon Choi, Xuechao Xing, Marcie A Glicksman, Gregory D Cuny, Elliot J Androphy, Kevin J Hodgetts
Spinal muscular atrophy (SMA) is the leading genetic cause of infant death. We previously developed a high-throughput assay that employs an SMN2-luciferase reporter allowing identification of compounds that act transcriptionally, enhance exon recognition, or stabilize the SMN protein. We describe optimization and characterization of an analog suitable for in vivo testing. Initially, we identified analog 4m that had good in vitro properties but low plasma and brain exposure in a mouse PK experiment due to short plasma stability; this was overcome by reversing the amide bond and changing the heterocycle...
May 19, 2017: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28474539/induced-neural-activity-promotes-an-oligodendroglia-regenerative-response-in-the-injured-spinal-cord-and-improves-motor-function-after-spinal-cord-injury
#16
Qun Li, Thierry Houdayer, Su Liu, Visar Belegu
Myelination in central nervous system (CNS) is a dynamic process that includes birth of oligodendrocyte progenitor cells (OPCs), their differentiation into oligodendrocytes, and ensheathment of axons. Regulation of myelination by neuronal activity has emerged as a new mechanism of CNS plasticity. Indeed, activity-dependent myelination has been shown to regulate sensory, motor and cognitive functions. In this work, we aimed to employ this mechanism of CNS plasticity by utilizing induced neuronal activity to promote remyelination and functional recovery in a subchronic model of SCI...
May 5, 2017: Journal of Neurotrauma
https://www.readbyqxmd.com/read/28468939/modelling-amyotrophic-lateral-sclerosis-progress-and-possibilities
#17
REVIEW
Philip Van Damme, Wim Robberecht, Ludo Van Den Bosch
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that primarily affects the motor system and presents with progressive muscle weakness. Most patients survive for only 2-5 years after disease onset, often due to failure of the respiratory muscles. ALS is a familial disease in ∼10% of patients, with the remaining 90% developing sporadic ALS. Over the past decade, major advances have been made in our understanding of the genetics and neuropathology of ALS. To date, around 20 genes are associated with ALS, with the most common causes of typical ALS associated with mutations in SOD1, TARDBP, FUS and C9orf72 Advances in our understanding of the genetic basis of ALS have led to the creation of different models of this disease...
May 1, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28466273/tdp-43-and-cytoskeletal-proteins-in-als
#18
REVIEW
Moritz Oberstadt, Joseph Claßen, Thomas Arendt, Max Holzer
Amyotrophic lateral sclerosis (ALS) represents a rapidly progressing neurodegenerative disease and is characterized by a degeneration of motor neurons. Motor neurons are particularly susceptible to selective and early degeneration because of their extended axon length and their dependency on the cytoskeleton for its stability, signaling, and axonal transport. The motor neuron cytoskeleton comprises actin filaments, neurofilaments like peripherin, and microtubules. The Transactivating Response Region (TAR) DNA Binding Protein (TDP-43) forms characteristic cytoplasmic aggregates in motor neurons of ALS patients, and at least in part, the pathogenesis of ALS seems to be driven by toxic pTDP-43 aggregates in cytoplasm, which lead to a diminished axon formation and reduced axon length...
May 2, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28466266/prolonged-dysfunction-of-astrocytes-and-activation-of-microglia-accelerate-degeneration-of-dopaminergic-neurons-in-the-rat-substantia-nigra-and-block-compensation-of-early-motor-dysfunction-induced-by-6-ohda
#19
Katarzyna Kuter, Łukasz Olech, Urszula Głowacka
Progressive degeneration of dopaminergic neurons in the substantia nigra (SN) is the underlying cause of Parkinson's disease (PD). The disease in early stages is difficult to diagnose, because behavioral deficits are masked by compensatory processes. Astrocytic and microglial pathology precedes motor symptoms. Besides supportive functions of astrocytes in the brain, their role in PD is unrecognized. Prolonged dysfunction of astrocytes could increase the vulnerability of dopaminergic neurons and advance their degeneration during aging...
May 2, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28466185/tissue-specific-transcription-of-the-neuronal-gene-lim3-affects-drosophila-melanogaster-lifespan-and-locomotion
#20
Olga Y Rybina, Svetlana V Sarantseva, Ekaterina R Veselkina, Olga I Bolschakova, Alexander V Symonenko, Anna V Krementsova, Elena V Ryabova, Natalia V Roshina, Elena G Pasyukova
The identity of neuronal cell types is established and maintained by the expression of neuronal genes coding for ion channels, neurotransmitters, and neuropeptides, among others. Some of these genes have been shown to affect lifespan; however, their role in lifespan control remains largely unclear. The Drosophila melanogaster gene Lim3 encodes a transcription factor involved in complicated motor neuron specification networks. We previously identified Lim3 as a candidate gene affecting lifespan. To obtain direct evidence of the involvement of Lim3 in lifespan control, Lim3 overexpression and RNAi knockdown were induced in the nervous system and muscles of Drosophila using the GAL4-UAS binary system...
May 2, 2017: Biogerontology
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