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Motor neuron development

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https://www.readbyqxmd.com/read/28729444/an-atoh1-s193a-phospho-mutant-allele-causes-hearing-deficits-and-motor-impairment
#1
Wei Rose Xie, Hsin-I Jen, Michelle L Seymour, Szu-Ying Yeh, Fred A Pereira, Andrew K Groves, Tiemo J Klisch, Huda Y Zoghbi
Atoh1 is a basic helix-loop-helix (bHLH) transcription factor that is essential for the genesis, survival, and maturation of a variety of neuronal and non-neuronal cell populations, including those involved in proprioception, interoception, balance, respiration, and hearing. Such diverse functions require fine regulation at the transcriptional and protein levels. Here we show that serine 193 (S193) is phosphorylated in Atoh1's bHLH domain in vivo Knock-in mice of both sexes bearing a GFP-tagged phospho-dead S193A allele on a null background (Atoh1(S193A/lacZ) ) exhibit mild cerebellar foliation defects, motor impairments, and also partial pontine nucleus migration defects, cochlear hair cell degeneration, and profound hearing loss...
July 20, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28726023/benefits-and-risks-of-non-approved-injection-regimens-for-botulinum-toxins-in-spasticity
#2
Andrea Santamato, Francesco Panza
Spasticity with muscle paresis and loss of dexterity is a common feature of upper motor neuron syndrome due to injuries or the pyramidal tract in several neurological conditions. Botulinum toxin type A has been considered the gold standard treatment for spasticity and movement disorders, with efficacy, reversibility, and low prevalence of complications. During the last 30 years, thousands of studies of its use have been performed, but few guidelines are available. Therefore, there is great variability in both the doses and intervals of administration and the approaches taken by clinicians with considerable experience in spasticity and movement disorder treatment...
July 19, 2017: Drugs
https://www.readbyqxmd.com/read/28724781/muscle-disuse-caused-by-botulinum-toxin-injection-leads-to-increased-central-gain-of-the-stretch-reflex-in-the-rat
#3
Jessica Pingel, Hans Hultborn, Lui Naslund-Koch, Dennis Bo Jensen, Jacob Wienecke, Jens Bo Nielsen
Botulinum toxin (Btx) is used in children with cerebral palsy and other neurological patients to diminish spasticity and reduce the risk of development of contractures. Here, we investigated changes in the central gain of the stretch reflex circuitry in response to botulinum toxin injection in the triceps surae muscle in rats. Experiments were performed in 21 rats. 8 rats were in a control group and 13 rats were injected with 6 IU of Btx in the left triceps surae muscle. Two weeks after Btx injection larger monosynaptic reflexes (MSR) were recorded from the left (injected) than the right (non-injected) L4 + L5 ventral roots following stimulation of the corresponding dorsal roots...
July 19, 2017: Journal of Neurophysiology
https://www.readbyqxmd.com/read/28720023/early-motor-development-and-cognitive-abilities-among-mexican-preschoolers
#4
Erika Osorio-Valencia, Luisa Torres-Sánchez, Lizbeth López-Carrillo, Stephen J Rothenberg, Lourdes Schnaas
Psychomotricity plays a very important role in children's development, especially for learning involving reading-writing and mathematical calculations. Evaluate motor development in children 3 years old and its relationship with their cognitive abilities at the age of 5 years. Based on a cohort study, we analyzed the information about motor performance evaluated at 3 years old by Peabody Motor Scale and cognitive abilities at 5 years old. The association was estimated using linear regression models adjusted by mother's intelligence quotient, sex, Bayley mental development index at 18 months, and quality of the environment at home (HOME scale)...
July 18, 2017: Child Neuropsychology: a Journal on Normal and Abnormal Development in Childhood and Adolescence
https://www.readbyqxmd.com/read/28714503/protective-effects-of-fisetin-and-other-berry-flavonoids-in-parkinson-s-disease
#5
REVIEW
Pamela Maher
Parkinson's disease (PD) is an age-associated degenerative disease of the midbrain that results from the loss of dopaminergic neurons in the substantia nigra. It initially presents as a movement disorder with cognitive and other behavioral problems appearing later in the progression of the disease. Current therapies for PD only delay the onset or reduce the motor symptoms. There are no treatments to stop the nerve cell death or to cure the disease. It is becoming increasingly clear that neurological diseases such as PD are multi-factorial involving disruptions in multiple cellular systems...
July 17, 2017: Food & Function
https://www.readbyqxmd.com/read/28711343/innexin-expression-in-electrically-coupled-motor-circuits
#6
REVIEW
Adriane Otopalik, Brian Lane, David J Schulz, Eve Marder
The many roles of innexins, the molecules that form gap junctions in invertebrates, have been explored in numerous species. Here, we present a summary of innexin expression and function in two small, central pattern generating circuits found in crustaceans: the stomatogastric ganglion and the cardiac ganglion. The two ganglia express multiple innexin genes, exhibit varying combinations of symmetrical and rectifying gap junctions, as well as gap junctions within and across different cell types. Past studies have revealed correlations in ion channel and innexin expression in coupled neurons, as well as intriguing functional relationships between ion channel conductances and electrical coupling...
July 12, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28709915/sex-differences-in-somatic-and-sensory-motor-development-after-neonatal-anoxia-in-wistar-rats
#7
Amrita Jha Kumar, Silvia Honda Takada, Lívia Clemente Motta-Teixeira, Vitor Yonamine Lee, Gilberto Fernando Xavier, Maria Inês Nogueira
Currently, one of the important causes of brain injury in new-borns is the neonatal anoxia which impacts the perinatology services worldwide. Animal models of anoxia have been used to assess its effects at cellular and behavioural levels in all ages, but few studies focus on sex differences. This study aimed to investigate some physical parameters of development, sensorimotor alterations, early neurological reflexes as well as the density of cells in motor and sensorimotor cerebral cortex of adolescent rats submitted to neonatal anoxia...
July 12, 2017: Behavioural Brain Research
https://www.readbyqxmd.com/read/28706504/hypertrophic-olivary-degeneration-and-palatal-or-oculopalatal-tremor
#8
REVIEW
Caroline Tilikete, Virginie Desestret
Hypertrophic degeneration of the inferior olive is mainly observed in patients developing palatal tremor (PT) or oculopalatal tremor (OPT). This syndrome manifests as a synchronous tremor of the palate (PT) and/or eyes (OPT) that may also involve other muscles from the branchial arches. It is associated with hypertrophic inferior olivary degeneration that is characterized by enlarged and vacuolated neurons, increased number and size of astrocytes, severe fibrillary gliosis, and demyelination. It appears on MRI as an increased T2/FLAIR signal intensity and enlargement of the inferior olive...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28705440/inhibition-of-trkb-at-the-nucleus-accumbens-using-ana-12-regulates-basal-and-stress-induced-orexin-a-expression-within-the-mesolimbic-system-and-affects-anxiety-sociability-and-motivation
#9
Idu Azogu, Helene Plamondon
Repeated stress exposure can lead to the development of anxiety and mood disorders. An emerging biological substrate of depression and associated pathology is the nucleus accumbens (NAc), which through interactions with limbic, cognitive and motor circuits can regulate a variety of stress responses. Within these circuits, orexin neurons are involved in arousal and stress adaptability, effects proposed mediated via brain-derived neurotrophic factor signaling. This study tested the hypotheses that 1) repeated exposure to heterotypic stress alters social ability and preference and passive avoidant behaviors, 2) TrkB receptors at the NAc shell regulates stress-induced behavioral responses and orexin expression within the mesocorticolimbic system...
July 10, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28704095/sarcopenia-an-endocrine-disorder
#10
Alexis McKee, John E Morley, Alvin M Matsumoto, Aaron Vinik
Sarcopenia is defined as low muscle function (walking speed or grip strength) in the presence of low muscle mass. A simple screening test - the SARC-F - is available to identify persons with sarcopenia. The major endocrine causes of sarcopenia are diabetes mellitus and male hypogonadism. Other causes are decreased physical activity, loss of motor neuron units, weight loss, inflammatory cytokines, reduced blood flow to muscles, very low 25(OH) vitamin D levels and decreased growth hormone and IGF-1. Treatment for sarcopenia includes resistance and aerobic exercise, leucine enriched essential amino acids, and vitamin D...
July 13, 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28701928/the-hyperpolarization-activated-current-determines-synaptic-excitability-calcium-activity-and-specific-viability-of-substantia-nigra-dopaminergic-neurons
#11
Carmen Carbone, Alessia Costa, Gustavo Provensi, Guido Mannaioni, Alessio Masi
Differential vulnerability between Substantia Nigra pars compacta (SNpc) and Ventral Tegmental Area (VTA) dopaminergic (DAergic) neurons is a hallmark of Parkinson's disease (PD). Understanding the molecular bases of this key histopathological aspect would foster the development of much-needed disease-modifying therapies. Non-heterogeneous DAergic degeneration is present in both toxin-based and genetic animal models, suggesting that cellular specificity, rather than causing factors, constitutes the background for differential vulnerability...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28701749/calcium-activated-sk-channels-control-firing-regularity-by-modulating-sodium-channel-availability-in-midbrain-dopamine-neurons
#12
Rajeshwari Iyer, Mark A Ungless, Aldo A Faisal
Dopamine neurons in the substantia nigra pars compacta and ventral tegmental area regulate behaviours such as reward-related learning, and motor control. Dysfunction of these neurons is implicated in Schizophrenia, addiction to drugs, and Parkinson's disease. While some dopamine neurons fire single spikes at regular intervals, others fire irregular single spikes interspersed with bursts. Pharmacological inhibition of calcium-activated potassium (SK) channels increases the variability in their firing pattern, sometimes also increasing the number of spikes fired in bursts, indicating that SK channels play an important role in maintaining dopamine neuron firing regularity and burst firing...
July 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28701747/neap-dusp26-suppresses-receptor-tyrosine-kinases-and-regulates-neuronal-development-in-zebrafish
#13
Chi-Hwa Yang, Yu-Jung Yeh, Jiz-Yuh Wang, Ya-Wen Liu, Yen-Lin Chen, Hui-Wen Cheng, Chun-Mei Cheng, Yung-Jen Chuang, Chiou-Hwa Yuh, Yi-Rong Chen
Expression of neuroendocrine-associated phosphatase (NEAP, also named as dual specificity phosphatase 26, [DUSP26]) is restricted to neuroendocrine tissues. We found that NEAP, but not its phosphatase-defective mutant, suppressed nerve growth factor (NGF) receptor TrkA and fibroblast growth factor receptor 1 (FGFR1) activation in PC12 cells upon NGF stimulation. Conversely, suppressing NEAP expression by RNA interference enhanced TrkA and FGFR1 phosphorylation. NEAP was capable of de-phosphorylating TrkA and FGFR1 directly in vitro...
July 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28699520/therapeutic-strategy-of-targeting-astrocytes-for-neuroprotection-in-parkinson-s-disease
#14
Ikuko Miyazaki, Masato Asanuma
Parkinson's disease (PD) is one of common neurodegenerative diseases, which shows motor symptoms including tremor, bradykinesia, rigidity and postural instability. It also involves non-motor symptoms such as cognitive impairment, mental manifestation, autonomic disorder and sensory disturbance. Although treatments to improve the motor disability in PD are being assessed at present, the main challenge remains that is the development of neuroprotective or disease-modifying treatments. Therefore, it is desirable to find approaches that can inhibit the progression of dopaminergic neurodegeneration...
July 10, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28698092/spasticity-in-multiple-sclerosis-contribution-of-inflammation-autoimmune-mediated-neuronal-damage-and-therapeutic-interventions
#15
REVIEW
Robert Patejdl, Uwe K Zettl
In contrast to other diseases that go along with spasticity (e.g. spinal cord injury), spasticity in chronic autoimmune diseases involving the CNS is complicated by the ongoing damage of neuronal networks that leads to permanent changes in the clinical picture of spasticity. Multiple sclerosis (MS) is the most frequent autoimmune disease of the central nervous system (CNS) and spasticity is one of the most disabling symptoms. It occurs in more than 80% MS patients at some point of the disease and is associated with impaired ambulation, pain and the development of contractures...
July 8, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28694434/laquinimod-treatment-in-the-r6-2-mouse-model
#16
Gisa Ellrichmann, Alina Blusch, Oluwaseun Fatoba, Janine Brunner, Liat Hayardeny, Michael Hayden, Dominik Sehr, Konstanze F Winklhofer, Carsten Saft, Ralf Gold
The transgenic mouse model R6/2 exhibits Huntington's disease (HD)-like deficits and basic pathophysiological similarities. We also used the pheochromocytoma-12 (PC12)-cell-line-model to investigate the effect of laquinimod on metabolic activity. Laquinimod is an orally administered immunomodulatory substance currently under development for the treatment of multiple sclerosis (MS) and HD. As an essential effect, increased levels of BDNF were observed. Therefore, we investigated the therapeutic efficacy of laquinimod in the R6/2 model, focusing on its neuroprotective capacity...
July 10, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28694016/-18-f-fdopa-positron-emission-tomography-in-manganese-exposed-workers
#17
Susan R Criswell, Susan Searles Nielsen, Mark Warden, Joel S Perlmutter, Stephen M Moerlein, Hubert P Flores, John Huang, Lianne Sheppard, Noah Seixas, Harvey Checkoway, Brad A Racette
Occupational manganese (Mn) exposure is associated with the development of parkinsonism; however, the mechanism of neurotoxicity is unknown. Brain positron emission tomography (PET) imaging provides a non-invasive method of assessing dopamineric neuronal function. 6-[(18)F]fluoro-L-DOPA (FDOPA) PET reflects in-vivo nigrostriatal function, but results in Mn exposure are conflicting. The objective of this study was to investigate the association between Mn exposure secondary to occupational welding, FDOPA striatal uptake, and clinical parkinsonism as measured by Unified Parkinson Disease Rating Scale motor subscore 3 (UPDRS3) scores...
July 7, 2017: Neurotoxicology
https://www.readbyqxmd.com/read/28687604/calpain-inhibition-is-protective-in-machado-joseph-disease-zebrafish-due-to-induction-of-autophagy
#18
Maxinne Watchon, Kristy C Yuan, Nick Mackovski, Adam J Svahn, Nicholas J Cole, Claire Goldsbury, Silke Rinkwitz, Thomas S Becker, Garth A Nicholson, Angela S Laird
The neurodegenerative disease Machado-Joseph disease (MJD), also known as spinocerebellar ataxin-3, affects neurons of the brain and spinal cord, disrupting control of the movement of muscles. We have successfully established the first transgenic zebrafish (Danio rerio) model of MJD, which express human ataxin-3 protein containing either 23 glutamines (23Q, wild-type) or 84Q (MJD-causing), within neurons. Phenotypic characterization of the zebrafish (male and female) revealed that the ataxin-3-84Q zebrafish have decreased survival compared to ataxin-3-23Q and develop ataxin-3 neuropathology, ataxin-3 cleavage fragments and motor impairment...
July 7, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28685918/a-long-term-bci-study-with-ecog-recordings-in-freely-moving-rats
#19
Thomas Costecalde, Tetiana Aksenova, Napoleon Torres-Martinez, Andriy Eliseyev, Corinne Mestais, Cecile Moro, Alim Louis Benabid
BACKGROUND: Brain Computer Interface (BCI) studies are performed in an increasing number of applications. Questions are raised about electrodes, data processing and effectors. Experiments are needed to solve these issues. OBJECTIVE: To develop a simple BCI set-up to easier studies for improving the mathematical tools to process the ECoG to control an effector. METHOD: We designed a simple BCI using transcranial electrodes (17 screws, three mechanically linked to create a common reference, 14 used as recording electrodes) to record Electro-Cortico-Graphic (ECoG) neuronal activities in rodents...
July 6, 2017: Neuromodulation: Journal of the International Neuromodulation Society
https://www.readbyqxmd.com/read/28684681/interplay-between-autophagy-exosomes-and-hiv-1-associated-neurological-disorders-new-insights-for-diagnosis-and-therapeutic-applications
#20
REVIEW
Chet Raj Ojha, Jessica Lapierre, Myosotys Rodriguez, Seth M Dever, Mohammad Asad Zadeh, Catherine DeMarino, Michelle L Pleet, Fatah Kashanchi, Nazira El-Hage
The autophagy-lysosomal pathway mediates a degradative process critical in the maintenance of cellular homeostasis as well as the preservation of proper organelle function by selective removal of damaged proteins and organelles. In some situations, cells remove unwanted or damaged proteins and RNAs through the release to the extracellular environment of exosomes. Since exosomes can be transferred from one cell to another, secretion of unwanted material to the extracellular environment in exosomes may have an impact, which can be beneficial or detrimental, in neighboring cells...
July 6, 2017: Viruses
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