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Motor neuron development

Guillermina López-Bendito
For the past two decades, we have advanced in our understanding of the mechanisms implicated in the formation of brain circuits. The connection between the cortex and thalamus has deserved much attention, as thalamocortical connectivity is crucial for sensory processing and motor learning. Classical dye tracing studies in wild-type and knockout mice initially helped to characterize the developmental progression of this connectivity and revealed key transcription factors involved. With the recent advances in technical tools to specifically label subsets of projecting neurons, knock-down genes individually and/or modify their activity, the field has gained further understanding on the rules operating in thalamocortical circuit formation and plasticity...
June 20, 2018: Neuroscience
Visou Ady, Brenda Toscano-Márquez, Moushumi Nath, Philip K Chang, Jeanette Hui, Anna Cook, François Charron, Roxanne Larivière, Bernard Brais, R Anne McKinney, Alanna J Watt
KEY POINTS: ARSACS is an early-onset neurodegenerative human disease characterized in part by ataxia and Purkinje cell loss in anterior cerebellar lobules. A knock-out mouse model has been developed that recapitulates several features of ARSACS. Using this ARSACS mouse model, we report changes in synaptic input and intrinsic firing in cerebellar Purkinje cells, as well as in their synaptic output in the DCN. Changes in firing are observed in anterior lobules that later exhibit Purkinje cell death, but not in posterior lobules that do not...
June 21, 2018: Journal of Physiology
Sara H Mokhtar, Min Joung Kim, Kylie A Magee, Pei Mun Aui, Speros Thomas, Maha M Bakhuraysah, Amani A Alrehaili, Jae Young Lee, David L Steer, Rachel Kenny, Catriona McLean, Michael F Azari, Antonis Birpanagos, Ewlina Lipiec, Philip Heraud, Bayden Wood, Steven Petratos
Alzheimer's disease (AD) is a neurodegenerative disorder characterized by accumulation of amyloid plaques and neurofibrillary tangles. Prior to the development of these characteristic pathological hallmarks of AD, anterograde axonal transport is impaired. However, the key proteins that initiate these intracellular impairments remain elusive. The collapsin response mediator protein-2 (CRMP-2) plays an integral role in kinesin-1-dependent axonal transport and there is evidence that phosphorylation of CRMP-2 releases kinesin-1...
June 2018: Neural Regeneration Research
Michael S Fernandopulle, Ryan Prestil, Christopher Grunseich, Chao Wang, Li Gan, Michael E Ward
Accurate modeling of human neuronal cell biology has been a long-standing challenge. However, methods to differentiate human induced pluripotent stem cells (iPSCs) to neurons have recently provided experimentally tractable cell models. Numerous methods that use small molecules to direct iPSCs into neuronal lineages have arisen in recent years. Unfortunately, these methods entail numerous challenges, including poor efficiency, variable cell type heterogeneity, and lengthy, expensive differentiation procedures...
June 2018: Current Protocols in Cell Biology
Tanuja Bordia, Xiomara Perez
L-dopa induced dyskinesias (LIDs) are a disabling motor complication of L-dopa therapy for Parkinson's disease (PD) management. Treatment options remain limited and the underlying network mechanisms remain unclear due to a complex pathophysiology. What is well-known, however, is that aberrant striatal signaling plays a key role in LIDs development. Here, we discuss the specific contribution of striatal cholinergic interneurons (ChIs) and GABAergic medium spiny projection neurons (MSNs) with a particular focus on how cholinergic signaling may integrate multiple striatal systems to modulate LIDs expression...
June 20, 2018: European Journal of Neuroscience
Jacob E Montgomery, Sarah Wahlstrom-Helgren, Timothy D Wiggin, Brittany M Corwin, Christina Lillesaar, Mark A Masino
Serotonin (5HT) is a modulator of many vital processes in the spinal cord (SC), such as production of locomotion. In the larval zebrafish, intraspinal serotonergic neurons (ISNs) are a source of spinal 5HT that, despite the availability of numerous genetic and optical tools, has not yet been directly shown to affect the spinal locomotor network. In order to better understand the functions of ISNs, we used a combination of strategies to investigate ISN development, morphology, and function. ISNs were optically isolated from one another by photoconverting Kaede fluorescent protein in individual cells, permitting morphometric analysis as they developed in vivo...
June 19, 2018: Developmental Neurobiology
Niamh Moriarty, Clare L Parish, Eilís Dowd
The dopamine precursor, levodopa, remains the 'gold-standard' treatment for Parkinson's disease, and, although it provides superlative efficacy in the early stages of the disease, its long-term use is limited by the development of severe motor side effects and a significant abating of therapeutic efficacy. Therefore, there remains a major unmet clinical need for the development of effective neuroprotective, neurorestorative or neuroreparatory therapies for this condition. The relatively selective loss of dopaminergic neurons from the nigrostriatal pathway makes Parkinson's disease an ideal candidate for reparative cell therapies wherein the dopaminergic neurons that are lost in the condition are replaced through direct cell transplantation into the brain...
June 20, 2018: European Journal of Neuroscience
Francisco Branoner, Hans Straka
Vestibulo-ocular reflexes (VORs) rely on neuronal computations that transform vestibular sensory signals into spatio-temporally appropriate extraocular motor commands. The motoneuronal discharge for contractions of the superior oblique eye muscle during linear translation derives from a utricular epithelial sector that is spatially aligned with the pulling direction of this muscle. In Xenopus laevis , the alignment is gradually achieved during larval development and requires motion-related semicircular canal afferent activity...
2018: Frontiers in Neurology
Cécile Baldy, Stéphanie Fournier, Samuel Boisjoly-Villeneuve, Marie-Ève Tremblay, Richard Kinkead
NEW FINDINGS: What is the central question of the study? Does neonatal stress, in the form of neonatal maternal separation, influence the maturation of microglial density, morphology, and neuronal signalling in medullary regions regulating cardio-respiratory function in rat pups? What is the main finding and its importance? Using Iba-1 immunohistochemistry, we show that neonatal maternal separation augments microglial density and the proportion of cells with an amoeboid morphology in the medulla...
June 19, 2018: Experimental Physiology
Emily W Baker, Holly A Kinder, Jessica M Hutcheson, Kylee Jo J Duberstein, Simon R Platt, Elizabeth W Howerth, Franklin D West
A number of preclinical rodent models have been developed in an effort to recapitulate injury mechanisms and identify potential therapeutics for traumatic brain injury (TBI), which is a major cause of death and long-term disability in the United States. However, the lack of restorative treatments for TBI has led to considerable criticism of current preclinical therapeutic development strategies, namely the translatability of widely-used rodent models to human patients. The use of large animal models, such as the pig, with more comparable brain anatomy and physiology to humans may enhance the translational capacity of current preclinical animal models...
June 19, 2018: Journal of Neurotrauma
Thomas Müller, Jan-Dominique Möhr
Parkinson's disease is a chronic, neurodegenerative disease. Its symptoms and course are heterogeneous. After several years of investigative drug studies, levodopa remains the most efficacious drug despite its long-term limitations. Consequently, research into new drug delivery modes is ongoing. Areas covered: This review summarizes past and current advances of levodopa therapy with a focus on long-term patient management. Current research aims to increase drug bioavailability and to deliver it to the brain continuously...
June 18, 2018: Expert Opinion on Pharmacotherapy
Pascual Torres, Omar Ramírez-Núñez, Ricardo Romero-Guevara, Gisel Barés, Ana B Granado-Serrano, Victòria Ayala, Jordi Boada, Laia Fontdevila, Monica Povedano, Daniel Sanchís, Reinald Pamplona, Isidro Ferrer, Manuel Portero-Otín
TARDBP (TAR DNA binding protein) is one of the components of neuronal aggregates in sporadic amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. We have developed a simple quantitative method to evaluate TARDBP splicing function that was applied to spinal cord, brainstem, motor cortex, and occipital cortex in ALS (n=8) cases compared to age- and gender-matched control (n=17). Then, we quantified the abundance of a TARDBP-spliced cryptic exon present in ATG4B (autophagy related 4B cysteine peptidase) mRNA...
June 18, 2018: Autophagy
Paulo José Lorenzoni, Renata Dal-Prá Ducci, Giuliano Ohde Dalledone, Claudia Suemi Kamoi Kay, Sérgio Monteiro de Almeida, Lineu Cesar Werneck, Rosana Hermínia Scola
HIV-associated motor neuron disease (MND), or amyotrophic lateral sclerosis (ALS)-like syndrome associated with HIV infection, is a rare manifestation of HIV infection. HIV-associated MND has only been identified in few cases to date. We analysed two Brazilian patients with HIV infection who developed MND. The diagnosis of HIV infection was concomitant with diagnosis of MND in one patient and it occurred eight years before the MND symptoms in another patient. The manifestation of MND in our patients with HIV infection was similar to classic ALS...
June 10, 2018: Clinical Neurology and Neurosurgery
Hayley S McLoughlin, Lauren R Moore, Ravi Chopra, Robert Komlo, Megan McKenzie, Kate G Blumenstein, Hien Zhao, Holly B Kordasiewicz, Vikram G Shakkottai, Henry L Paulson
OBJECTIVE: Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease, is the most common dominantly inherited ataxia. Despite advances in understanding this CAG repeat/polyglutamine expansion disease, there are still no therapies to alter its progressive fatal course. Here we investigate whether an antisense oligonucleotide (ASO) targeting the SCA3 disease gene, ATXN3, can prevent molecular, neuropathological, electrophysiological and behavioral features of the disease in a mouse model of SCA3...
June 16, 2018: Annals of Neurology
Giulia Costa, Annalisa Pinna, Pier Francesca Porceddu, Maria Antonietta Casu, Anna Di Maio, Francesco Napolitano, Alessandro Usiello, Micaela Morelli
We have recently shown that male Rhes knockout (KO) mice develop a mild form of spontaneous Parkinson's disease (PD)-like phenotype, characterized by motor impairment and a decrease in nigrostriatal dopamine (DA) neurons. Experimental evidence has implicated neuroinflammation in PD progression, and the presence of activated glial cells has been correlated with DA neuron degeneration. Despite this, several factors, such as gender, have been found to affect DAergic neuron degeneration and influence neuroinflammation, explaining the differences between men and women in the etiology of PD...
2018: Frontiers in Aging Neuroscience
Sarah Hargreaves, Peter A Bath, Suzanne Duffin, Julie Ellis
BACKGROUND: The availability of an increasing number of online health forums has altered the experience of living with a health condition, as more people are now able to connect and support one another. Empathy is an important component of peer-to-peer support, although little is known about how empathy develops and operates within online health forums. OBJECTIVE: The aim of this paper is to explore how empathy develops and operates within two online health forums for differing health conditions: breast cancer and motor neuron disease (MND), also known as amyotrophic lateral sclerosis...
June 14, 2018: Journal of Medical Internet Research
Serena Stanga, Liliana Brambilla, Bernadette Tasiaux, Anh H Dang, Adrian Ivanoiu, Jean-Noël Octave, Daniela Rossi, Vincent van Pesch, Pascal Kienlen-Campard
The current inability of clinical criteria to accurately identify the "at-risk group" for Amyotrophic Lateral Sclerosis (ALS) development as well as its unknown etiology are fueling the interest in biomarkers aimed at completing clinical approaches for the diagnosis. The Glial cell line-derived neurotrophic factor (GDNF) is a diffusible peptide critically involved in neuronal differentiation and survival. GDNF is largely studied in various neurological and neuromuscular diseases, with a great interest in the peripheral nervous system (PNS)...
2018: Frontiers in Neurology
Akanksha Mishra, Sonu Singh, Shubha Shukla
Dopamine controls various physiological functions in the brain and periphery by acting on its receptors D1, D2, D3, D4, and D5. Dopamine receptors are G protein-coupled receptors involved in the regulation of motor activity and several neurological disorders such as schizophrenia, bipolar disorder, Parkinson's disease (PD), Alzheimer's disease, and attention-deficit/hyperactivity disorder. Reduction in dopamine content in the nigrostriatal pathway is associated with the development of PD, along with the degeneration of dopaminergic neurons in the substantia nigra region...
2018: Journal of Experimental Neuroscience
Camila Nascimento Alves, Tiago Kiyoshi Kitabayashi Braga, Danusa Neves Somensi, Bruno Sérgio Vilhena do Nascimento, José Antônio Santos de Lima, Satomi Fujihara
The X-linked spinal and bulbar muscular atrophy (Kennedy's disease) is a rare X-linked, recessive, lower motor neuron disease, characterized by weakness, atrophy, and fasciculations of the appendicular and bulbar muscle. The disease is caused by an expansion of the CAG repetition in the androgen receptor gene. Patients with Kennedy's disease have more than 39 CAG repetitions. We report a case of 57-year-old man, resident of Monte Dourado (PA, Brazil) who complained of brachiocrural paresis evolving for 3 years along with fasciculations and tremors of extremities...
June 7, 2018: Einstein
Auriane Corbière, Marie-Laure Walet-Balieu, Philippe Chan, Magali Basille-Dugay, Julie Hardouin, David Vaudry
The cerebellum is a brain structure involved in motor and cognitive functions. The development of the cerebellar cortex (the external part of the cerebellum) is under the control of numerous factors. Among these factors, neuropeptides including PACAP or somatostatin modulate the survival, migration and/or differentiation of cerebellar granule cells. Interestingly, such peptides contributing to cerebellar ontogenesis usually exhibit a specific transient expression profile with a low abundance at birth, a high expression level during the developmental processes, which take place within the first two postnatal weeks in rodents, and a gradual decline toward adulthood...
June 12, 2018: Molecular & Cellular Proteomics: MCP
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