Motor Neuron Disease: Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex. Clinical subtypes are distinguished by the major site of degeneration. In AMYOTROPHIC LATERAL SCLEROSIS there is involvement of upper, lower, and brainstem motor neurons. In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are primarily affected. With progressive bulbar palsy (BULBAR PALSY, PROGRESSIVE), the initial degeneration occurs in the brainstem. In primary lateral sclerosis, the cortical neurons are affected in isolation. (Adams et al., Principles of Neurology, 6th ed, p1089)Motor Neurons: Neurons which activate MUSCLE CELLS.Amyotrophic Lateral Sclerosis: A degenerative disorder affecting upper MOTOR NEURONS in the brain and lower motor neurons in the brain stem and SPINAL CORD. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, FASCICULATION, hyperreflexia, DYSARTHRIA, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous ASTROCYTES and atrophy of anterior SPINAL NERVE ROOTS and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94)Superoxide Dismutase: An oxidoreductase that catalyzes the reaction between superoxide anions and hydrogen to yield molecular oxygen and hydrogen peroxide. The enzyme protects the cell against dangerous levels of superoxide. EC 184.108.40.206.Neurons: The basic cellular units of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the NERVOUS SYSTEM.Axons: Nerve fibers that are capable of rapidly conducting impulses away from the neuron cell body.Survival of Motor Neuron 1 Protein: A SMN complex protein that is essential for the function of the SMN protein complex. In humans the protein is encoded by a single gene found near the inversion telomere of a large inverted region of CHROMOSOME 5. Mutations in the gene coding for survival of motor neuron 1 protein may result in SPINAL MUSCULAR ATROPHIES OF CHILDHOOD.Neurons, Afferent: Neurons which conduct NERVE IMPULSES to the CENTRAL NERVOUS SYSTEM.Motor Cortex: Area of the FRONTAL LOBE concerned with primary motor control located in the dorsal PRECENTRAL GYRUS immediately anterior to the central sulcus. It is comprised of three areas: the primary motor cortex located on the anterior paracentral lobule on the medial surface of the brain; the premotor cortex located anterior to the primary motor cortex; and the supplementary motor area located on the midline surface of the hemisphere anterior to the primary motor cortex.Survival of Motor Neuron 2 Protein: A SMN complex protein that is closely-related to SURVIVAL OF MOTOR NEURON 1 PROTEIN. In humans, the protein is encoded by an often duplicated gene found near the inversion centromere of a large inverted region of CHROMOSOME 5.Mutation: Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.Evoked Potentials, Motor: The electrical response evoked in a muscle or motor nerve by electrical or magnetic stimulation. Common methods of stimulation are by transcranial electrical and TRANSCRANIAL MAGNETIC STIMULATION. It is often used for monitoring during neurosurgery.Spinal Cord: A cylindrical column of tissue that lies within the vertebral canal. It is composed of WHITE MATTER and GRAY MATTER.Muscular Atrophy, Spinal: A group of disorders marked by progressive degeneration of motor neurons in the spinal cord resulting in weakness and muscular atrophy, usually without evidence of injury to the corticospinal tracts. Diseases in this category include Werdnig-Hoffmann disease and later onset SPINAL MUSCULAR ATROPHIES OF CHILDHOOD, most of which are hereditary. (Adams et al., Principles of Neurology, 6th ed, p1089)Motor Activity: The physical activity of a human or an animal as a behavioral phenomenon.SMN Complex Proteins: A complex of proteins that assemble the SNRNP CORE PROTEINS into a core structure that surrounds a highly conserved RNA sequence found in SMALL NUCLEAR RNA. They are found localized in the GEMINI OF COILED BODIES and in the CYTOPLASM. The SMN complex is named after the Survival of Motor Neuron Complex Protein 1, which is a critical component of the complex.Action Potentials: Abrupt changes in the membrane potential that sweep along the CELL MEMBRANE of excitable cells in response to excitation stimuli.Nerve Tissue ProteinsMolecular Motor Proteins: Proteins that are involved in or cause CELL MOVEMENT such as the rotary structures (flagellar motor) or the structures whose movement is directed along cytoskeletal filaments (MYOSIN; KINESIN; and DYNEIN motor families).Electric Stimulation: Use of electric potential or currents to elicit biological responses.Nerve Degeneration: Loss of functional activity and trophic degeneration of nerve axons and their terminal arborizations following the destruction of their cells of origin or interruption of their continuity with these cells. The pathology is characteristic of neurodegenerative diseases. Often the process of nerve degeneration is studied in research on neuroanatomical localization and correlation of the neurophysiology of neural pathways.Synapses: Specialized junctions at which a neuron communicates with a target cell. At classical synapses, a neuron's presynaptic terminal releases a chemical transmitter stored in synaptic vesicles which diffuses across a narrow synaptic cleft and activates receptors on the postsynaptic membrane of the target cell. The target may be a dendrite, cell body, or axon of another neuron, or a specialized region of a muscle or secretory cell. Neurons may also communicate via direct electrical coupling with ELECTRICAL SYNAPSES. Several other non-synaptic chemical or electric signal transmitting processes occur via extracellular mediated interactions.Neuromuscular Diseases: A general term encompassing lower MOTOR NEURON DISEASE; PERIPHERAL NERVOUS SYSTEM DISEASES; and certain MUSCULAR DISEASES. Manifestations include MUSCLE WEAKNESS; FASCICULATION; muscle ATROPHY; SPASM; MYOKYMIA; MUSCLE HYPERTONIA, myalgias, and MUSCLE HYPOTONIA.Ganglia, Invertebrate: Clusters of neuronal cell bodies in invertebrates. Invertebrate ganglia may also contain neuronal processes and non-neuronal supporting cells. Many invertebrate ganglia are favorable subjects for research because they have small numbers of functional neuronal types which can be identified from one animal to another.Electrophysiology: The study of the generation and behavior of electrical charges in living organisms particularly the nervous system and the effects of electricity on living organisms.Interneurons: Most generally any NEURONS which are not motor or sensory. Interneurons may also refer to neurons whose AXONS remain within a particular brain region in contrast to projection neurons, which have axons projecting to other brain regions.Synaptic Transmission: The communication from a NEURON to a target (neuron, muscle, or secretory cell) across a SYNAPSE. In chemical synaptic transmission, the presynaptic neuron releases a NEUROTRANSMITTER that diffuses across the synaptic cleft and binds to specific synaptic receptors, activating them. The activated receptors modulate specific ion channels and/or second-messenger systems in the postsynaptic cell. In electrical synaptic transmission, electrical signals are communicated as an ionic current flow across ELECTRICAL SYNAPSES.Movement: The act, process, or result of passing from one place or position to another. It differs from LOCOMOTION in that locomotion is restricted to the passing of the whole body from one place to another, while movement encompasses both locomotion but also a change of the position of the whole body or any of its parts. Movement may be used with reference to humans, vertebrate and invertebrate animals, and microorganisms. Differentiate also from MOTOR ACTIVITY, movement associated with behavior.Cholinergic Neurons: Neurons whose primary neurotransmitter is ACETYLCHOLINE.Cells, Cultured: Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.Aplysia: An opisthobranch mollusk of the order Anaspidea. It is used frequently in studies of nervous system development because of its large identifiable neurons. Aplysiatoxin and its derivatives are not biosynthesized by Aplysia, but acquired by ingestion of Lyngbya (seaweed) species.Mice, Transgenic: Laboratory mice that have been produced from a genetically manipulated EGG or EMBRYO, MAMMALIAN.Dopaminergic Neurons: Neurons whose primary neurotransmitter is DOPAMINE.Electromyography: Recording of the changes in electric potential of muscle by means of surface or needle electrodes.Anterior Horn Cells: MOTOR NEURONS in the anterior (ventral) horn of the SPINAL CORD which project to SKELETAL MUSCLES.Neuromuscular Junction: The synapse between a neuron and a muscle.Dendrites: Extensions of the nerve cell body. They are short and branched and receive stimuli from other NEURONS.Sensory Receptor Cells: Specialized afferent neurons capable of transducing sensory stimuli into NERVE IMPULSES to be transmitted to the CENTRAL NERVOUS SYSTEM. Sometimes sensory receptors for external stimuli are called exteroceptors; for internal stimuli are called interoceptors and proprioceptors.Bulbar Palsy, Progressive: A motor neuron disease marked by progressive weakness of the muscles innervated by cranial nerves of the lower brain stem. Clinical manifestations include dysarthria, dysphagia, facial weakness, tongue weakness, and fasciculations of the tongue and facial muscles. The adult form of the disease is marked initially by bulbar weakness which progresses to involve motor neurons throughout the neuroaxis. Eventually this condition may become indistinguishable from AMYOTROPHIC LATERAL SCLEROSIS. Fazio-Londe syndrome is an inherited form of this illness which occurs in children and young adults. (Adams et al., Principles of Neurology, 6th ed, p1091; Brain 1992 Dec;115(Pt 6):1889-1900)Ganglia, Spinal: Sensory ganglia located on the dorsal spinal roots within the vertebral column. The spinal ganglion cells are pseudounipolar. The single primary branch bifurcates sending a peripheral process to carry sensory information from the periphery and a central branch which relays that information to the spinal cord or brain.Neural Inhibition: The function of opposing or restraining the excitation of neurons or their target excitable cells.GABAergic Neurons: Neurons whose primary neurotransmitter is GAMMA-AMINOBUTYRIC ACID.Patch-Clamp Techniques: An electrophysiologic technique for studying cells, cell membranes, and occasionally isolated organelles. All patch-clamp methods rely on a very high-resistance seal between a micropipette and a membrane; the seal is usually attained by gentle suction. The four most common variants include on-cell patch, inside-out patch, outside-out patch, and whole-cell clamp. Patch-clamp methods are commonly used to voltage clamp, that is control the voltage across the membrane and measure current flow, but current-clamp methods, in which the current is controlled and the voltage is measured, are also used.Immunohistochemistry: Histochemical localization of immunoreactive substances using labeled antibodies as reagents.Models, Neurological: Theoretical representations that simulate the behavior or activity of the neurological system, processes or phenomena; includes the use of mathematical equations, computers, and other electronic equipment.Brain: The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.Cerebral Cortex: The thin layer of GRAY MATTER on the surface of the CEREBRAL HEMISPHERES that develops from the TELENCEPHALON and folds into gyri and sulchi. It reaches its highest development in humans and is responsible for intellectual faculties and higher mental functions.Animals, Newborn: Refers to animals in the period of time just after birth.Disease Models, Animal: Naturally occurring or experimentally induced animal diseases with pathological processes sufficiently similar to those of human diseases. They are used as study models for human diseases.Time Factors: Elements of limited time intervals, contributing to particular results or situations.Facial Nerve: The 7th cranial nerve. The facial nerve has two parts, the larger motor root which may be called the facial nerve proper, and the smaller intermediate or sensory root. Together they provide efferent innervation to the muscles of facial expression and to the lacrimal and SALIVARY GLANDS, and convey afferent information for TASTE from the anterior two-thirds of the TONGUE and for TOUCH from the EXTERNAL EAR.Membrane Potentials: The voltage differences across a membrane. For cellular membranes they are computed by subtracting the voltage measured outside the membrane from the voltage measured inside the membrane. They result from differences of inside versus outside concentration of potassium, sodium, chloride, and other ions across cells' or ORGANELLES membranes. For excitable cells, the resting membrane potentials range between -30 and -100 millivolts. Physical, chemical, or electrical stimuli can make a membrane potential more negative (hyperpolarization), or less negative (depolarization).Motor Skills Disorders: Marked impairments in the development of motor coordination such that the impairment interferes with activities of daily living. (From DSM-V)Animals, Genetically Modified: ANIMALS whose GENOME has been altered by GENETIC ENGINEERING, or their offspring.Hippocampus: A curved elevation of GRAY MATTER extending the entire length of the floor of the TEMPORAL HORN of the LATERAL VENTRICLE (see also TEMPORAL LOBE). The hippocampus proper, subiculum, and DENTATE GYRUS constitute the hippocampal formation. Sometimes authors include the ENTORHINAL CORTEX in the hippocampal formation.gamma-Aminobutyric Acid: The most common inhibitory neurotransmitter in the central nervous system.Brain Stem: The part of the brain that connects the CEREBRAL HEMISPHERES with the SPINAL CORD. It consists of the MESENCEPHALON; PONS; and MEDULLA OBLONGATA.Axonal Transport: The directed transport of ORGANELLES and molecules along nerve cell AXONS. Transport can be anterograde (from the cell body) or retrograde (toward the cell body). (Alberts et al., Molecular Biology of the Cell, 3d ed, pG3)Neuropeptides: Peptides released by NEURONS as intercellular messengers. Many neuropeptides are also hormones released by non-neuronal cells.Neural Conduction: The propagation of the NERVE IMPULSE along the nerve away from the site of an excitation stimulus.Choline O-Acetyltransferase: An enzyme that catalyzes the formation of acetylcholine from acetyl-CoA and choline. EC 220.127.116.11.Ganglia: Clusters of multipolar neurons surrounded by a capsule of loosely organized CONNECTIVE TISSUE located outside the CENTRAL NERVOUS SYSTEM.Grasshoppers: Plant-eating orthopterans having hindlegs adapted for jumping. There are two main families: Acrididae and Romaleidae. Some of the more common genera are: Melanoplus, the most common grasshopper; Conocephalus, the eastern meadow grasshopper; and Pterophylla, the true katydid.Neurons, Efferent: Neurons which send impulses peripherally to activate muscles or secretory cells.Nerve Net: A meshlike structure composed of interconnecting nerve cells that are separated at the synaptic junction or joined to one another by cytoplasmic processes. In invertebrates, for example, the nerve net allows nerve impulses to spread over a wide area of the net because synapses can pass information in any direction.Neurofilament Proteins: Type III intermediate filament proteins that assemble into neurofilaments, the major cytoskeletal element in nerve axons and dendrites. They consist of three distinct polypeptides, the neurofilament triplet. Types I, II, and IV intermediate filament proteins form other cytoskeletal elements such as keratins and lamins. It appears that the metabolism of neurofilaments is disturbed in Alzheimer's disease, as indicated by the presence of neurofilament epitopes in the neurofibrillary tangles, as well as by the severe reduction of the expression of the gene for the light neurofilament subunit of the neurofilament triplet in brains of Alzheimer's patients. (Can J Neurol Sci 1990 Aug;17(3):302)Locomotion: Movement or the ability to move from one place or another. It can refer to humans, vertebrate or invertebrate animals, and microorganisms.Central Nervous System: The main information-processing organs of the nervous system, consisting of the brain, spinal cord, and meninges.Reaction Time: The time from the onset of a stimulus until a response is observed.Green Fluorescent Proteins: Protein analogs and derivatives of the Aequorea victoria green fluorescent protein that emit light (FLUORESCENCE) when excited with ULTRAVIOLET RAYS. They are used in REPORTER GENES in doing GENETIC TECHNIQUES. Numerous mutants have been made to emit other colors or be sensitive to pH.Behavior, Animal: The observable response an animal makes to any situation.Medulla Oblongata: The lower portion of the BRAIN STEM. It is inferior to the PONS and anterior to the CEREBELLUM. Medulla oblongata serves as a relay station between the brain and the spinal cord, and contains centers for regulating respiratory, vasomotor, cardiac, and reflex activities.Excitatory Postsynaptic Potentials: Depolarization of membrane potentials at the SYNAPTIC MEMBRANES of target neurons during neurotransmission. Excitatory postsynaptic potentials can singly or in summation reach the trigger threshold for ACTION POTENTIALS.Neuronal Plasticity: The capacity of the NERVOUS SYSTEM to change its reactivity as the result of successive activations.Evoked Potentials: Electrical responses recorded from nerve, muscle, SENSORY RECEPTOR, or area of the CENTRAL NERVOUS SYSTEM following stimulation. They range from less than a microvolt to several microvolts. The evoked potential can be auditory (EVOKED POTENTIALS, AUDITORY), somatosensory (EVOKED POTENTIALS, SOMATOSENSORY), visual (EVOKED POTENTIALS, VISUAL), or motor (EVOKED POTENTIALS, MOTOR), or other modalities that have been reported.Cell Count: The number of CELLS of a specific kind, usually measured per unit volume or area of sample.Mice, Inbred C57BLMuscle, Skeletal: A subtype of striated muscle, attached by TENDONS to the SKELETON. Skeletal muscles are innervated and their movement can be consciously controlled. They are also called voluntary muscles.Gene Expression Regulation, Developmental: Any of the processes by which nuclear, cytoplasmic, or intercellular factors influence the differential control of gene action during the developmental stages of an organism.Olfactory Receptor Neurons: Neurons in the OLFACTORY EPITHELIUM with proteins (RECEPTORS, ODORANT) that bind, and thus detect, odorants. These neurons send their DENDRITES to the surface of the epithelium with the odorant receptors residing in the apical non-motile cilia. Their unmyelinated AXONS synapse in the OLFACTORY BULB of the BRAIN.Glutamic Acid: A non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM.Psychomotor Performance: The coordination of a sensory or ideational (cognitive) process and a motor activity.Cats: The domestic cat, Felis catus, of the carnivore family FELIDAE, comprising over 30 different breeds. The domestic cat is descended primarily from the wild cat of Africa and extreme southwestern Asia. Though probably present in towns in Palestine as long ago as 7000 years, actual domestication occurred in Egypt about 4000 years ago. (From Walker's Mammals of the World, 6th ed, p801)Afferent Pathways: Nerve structures through which impulses are conducted from a peripheral part toward a nerve center.Dopamine: One of the catecholamine NEUROTRANSMITTERS in the brain. It is derived from TYROSINE and is the precursor to NOREPINEPHRINE and EPINEPHRINE. Dopamine is a major transmitter in the extrapyramidal system of the brain, and important in regulating movement. A family of receptors (RECEPTORS, DOPAMINE) mediate its action.Leeches: Annelids of the class Hirudinea. Some species, the bloodsuckers, may become temporarily parasitic upon animals, including man. Medicinal leeches (HIRUDO MEDICINALIS) have been used therapeutically for drawing blood since ancient times.Cyclic AMP Response Element-Binding Protein: A protein that has been shown to function as a calcium-regulated transcription factor as well as a substrate for depolarization-activated CALCIUM-CALMODULIN-DEPENDENT PROTEIN KINASES. This protein functions to integrate both calcium and cAMP signals.Chick Embryo: The developmental entity of a fertilized chicken egg (ZYGOTE). The developmental process begins about 24 h before the egg is laid at the BLASTODISC, a small whitish spot on the surface of the EGG YOLK. After 21 days of incubation, the embryo is fully developed before hatching.Rats, Sprague-Dawley: A strain of albino rat used widely for experimental purposes because of its calmness and ease of handling. It was developed by the Sprague-Dawley Animal Company.Muscles: Contractile tissue that produces movement in animals.Neuroglia: The non-neuronal cells of the nervous system. They not only provide physical support, but also respond to injury, regulate the ionic and chemical composition of the extracellular milieu, participate in the BLOOD-BRAIN BARRIER and BLOOD-RETINAL BARRIER, form the myelin insulation of nervous pathways, guide neuronal migration during development, and exchange metabolites with neurons. Neuroglia have high-affinity transmitter uptake systems, voltage-dependent and transmitter-gated ion channels, and can release transmitters, but their role in signaling (as in many other functions) is unclear.Rhombencephalon: The posterior of the three primitive cerebral vesicles of an embryonic brain. It consists of myelencephalon, metencephalon, and isthmus rhombencephali from which develop the major BRAIN STEM components, such as MEDULLA OBLONGATA from the myelencephalon, CEREBELLUM and PONS from the metencephalon, with the expanded cavity forming the FOURTH VENTRICLE.Paralysis: A general term most often used to describe severe or complete loss of muscle strength due to motor system disease from the level of the cerebral cortex to the muscle fiber. This term may also occasionally refer to a loss of sensory function. (From Adams et al., Principles of Neurology, 6th ed, p45)Analysis of Variance: A statistical technique that isolates and assesses the contributions of categorical independent variables to variation in the mean of a continuous dependent variable.Pyramidal Cells: Projection neurons in the CEREBRAL CORTEX and the HIPPOCAMPUS. Pyramidal cells have a pyramid-shaped soma with the apex and an apical dendrite pointed toward the pial surface and other dendrites and an axon emerging from the base. The axons may have local collaterals but also project outside their cortical region.Spinal Muscular Atrophies of Childhood: A group of recessively inherited diseases that feature progressive muscular atrophy and hypotonia. They are classified as type I (Werdnig-Hoffman disease), type II (intermediate form), and type III (Kugelberg-Welander disease). Type I is fatal in infancy, type II has a late infantile onset and is associated with survival into the second or third decade. Type III has its onset in childhood, and is slowly progressive. (J Med Genet 1996 Apr:33(4):281-3)Rats, Wistar: A strain of albino rat developed at the Wistar Institute that has spread widely at other institutions. This has markedly diluted the original strain.Cell Differentiation: Progressive restriction of the developmental potential and increasing specialization of function that leads to the formation of specialized cells, tissues, and organs.Cerebellum: The part of brain that lies behind the BRAIN STEM in the posterior base of skull (CRANIAL FOSSA, POSTERIOR). It is also known as the "little brain" with convolutions similar to those of CEREBRAL CORTEX, inner white matter, and deep cerebellar nuclei. Its function is to coordinate voluntary movements, maintain balance, and learn motor skills.Astrocytes: A class of large neuroglial (macroglial) cells in the central nervous system - the largest and most numerous neuroglial cells in the brain and spinal cord. Astrocytes (from "star" cells) are irregularly shaped with many long processes, including those with "end feet" which form the glial (limiting) membrane and directly and indirectly contribute to the BLOOD-BRAIN BARRIER. They regulate the extracellular ionic and chemical environment, and "reactive astrocytes" (along with MICROGLIA) respond to injury.Neurogenesis: Formation of NEURONS which involves the differentiation and division of STEM CELLS in which one or both of the daughter cells become neurons.Periodicity: The tendency of a phenomenon to recur at regular intervals; in biological systems, the recurrence of certain activities (including hormonal, cellular, neural) may be annual, seasonal, monthly, daily, or more frequently (ultradian).Nerve Growth Factors: Factors which enhance the growth potentialities of sensory and sympathetic nerve cells.Mice, Knockout: Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.Physical Stimulation: Act of eliciting a response from a person or organism through physical contact.Mesencephalon: The middle of the three primitive cerebral vesicles of the embryonic brain. Without further subdivision, midbrain develops into a short, constricted portion connecting the PONS and the DIENCEPHALON. Midbrain contains two major parts, the dorsal TECTUM MESENCEPHALI and the ventral TEGMENTUM MESENCEPHALI, housing components of auditory, visual, and other sensorimoter systems.Peripheral Nerves: The nerves outside of the brain and spinal cord, including the autonomic, cranial, and spinal nerves. Peripheral nerves contain non-neuronal cells and connective tissue as well as axons. The connective tissue layers include, from the outside to the inside, the epineurium, the perineurium, and the endoneurium.FMRFamide: A molluscan neuroactive peptide which induces a fast excitatory depolarizing response due to direct activation of amiloride-sensitive SODIUM CHANNELS. (From Nature 1995; 378(6558): 730-3)Serotonin: A biochemical messenger and regulator, synthesized from the essential amino acid L-TRYPTOPHAN. In humans it is found primarily in the central nervous system, gastrointestinal tract, and blood platelets. Serotonin mediates several important physiological functions including neurotransmission, gastrointestinal motility, hemostasis, and cardiovascular integrity. Multiple receptor families (RECEPTORS, SEROTONIN) explain the broad physiological actions and distribution of this biochemical mediator.Nerve Regeneration: Renewal or physiological repair of damaged nerve tissue.Neurites: In tissue culture, hairlike projections of neurons stimulated by growth factors and other molecules. These projections may go on to form a branched tree of dendrites or a single axon or they may be reabsorbed at a later stage of development. "Neurite" may refer to any filamentous or pointed outgrowth of an embryonal or tissue-culture neural cell.Cell Death: The termination of the cell's ability to carry out vital functions such as metabolism, growth, reproduction, responsiveness, and adaptability.Kinesin: A microtubule-associated mechanical adenosine triphosphatase, that uses the energy of ATP hydrolysis to move organelles along microtubules toward the plus end of the microtubule. The protein is found in squid axoplasm, optic lobes, and in bovine brain. Bovine kinesin is a heterotetramer composed of two heavy (120 kDa) and two light (62 kDa) chains. EC 3.6.1.-.Axotomy: Transection or severing of an axon. This type of denervation is used often in experimental studies on neuronal physiology and neuronal death or survival, toward an understanding of nervous system disease.Cell Survival: The span of viability of a cell characterized by the capacity to perform certain functions such as metabolism, growth, reproduction, some form of responsiveness, and adaptability.Signal Transduction: The intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GAMMA-AMINOBUTYRIC ACID-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptor-mediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway.Functional Laterality: Behavioral manifestations of cerebral dominance in which there is preferential use and superior functioning of either the left or the right side, as in the preferred use of the right hand or right foot.Recruitment, Neurophysiological: The spread of response if stimulation is prolonged. (Campbell's Psychiatric Dictionary, 8th ed.)Nervous System: The entire nerve apparatus, composed of a central part, the brain and spinal cord, and a peripheral part, the cranial and spinal nerves, autonomic ganglia, and plexuses. (Stedman, 26th ed)Tetrodotoxin: An aminoperhydroquinazoline poison found mainly in the liver and ovaries of fishes in the order TETRAODONTIFORMES, which are eaten. The toxin causes paresthesia and paralysis through interference with neuromuscular conduction.Cranial Nerves: Twelve pairs of nerves that carry general afferent, visceral afferent, special afferent, somatic efferent, and autonomic efferent fibers.Caenorhabditis elegans: A species of nematode that is widely used in biological, biochemical, and genetic studies.Homeodomain Proteins: Proteins encoded by homeobox genes (GENES, HOMEOBOX) that exhibit structural similarity to certain prokaryotic and eukaryotic DNA-binding proteins. Homeodomain proteins are involved in the control of gene expression during morphogenesis and development (GENE EXPRESSION REGULATION, DEVELOPMENTAL).In Situ Hybridization: A technique that localizes specific nucleic acid sequences within intact chromosomes, eukaryotic cells, or bacterial cells through the use of specific nucleic acid-labeled probes.Presynaptic Terminals: The distal terminations of axons which are specialized for the release of neurotransmitters. Also included are varicosities along the course of axons which have similar specializations and also release transmitters. Presynaptic terminals in both the central and peripheral nervous systems are included.Sciatic Nerve: A nerve which originates in the lumbar and sacral spinal cord (L4 to S3) and supplies motor and sensory innervation to the lower extremity. The sciatic nerve, which is the main continuation of the sacral plexus, is the largest nerve in the body. It has two major branches, the TIBIAL NERVE and the PERONEAL NERVE.Zebrafish: An exotic species of the family CYPRINIDAE, originally from Asia, that has been introduced in North America. They are used in embryological studies and to study the effects of certain chemicals on development.RNA-Binding Proteins: Proteins that bind to RNA molecules. Included here are RIBONUCLEOPROTEINS and other proteins whose function is to bind specifically to RNA.Serotonergic Neurons: Neurons whose primary neurotransmitter is SEROTONIN.Astacoidea: A superfamily of various freshwater CRUSTACEA, in the infraorder Astacidea, comprising the crayfish. Common genera include Astacus and Procambarus. Crayfish resemble lobsters, but are usually much smaller.Muscle Contraction: A process leading to shortening and/or development of tension in muscle tissue. Muscle contraction occurs by a sliding filament mechanism whereby actin filaments slide inward among the myosin filaments.Calcium: A basic element found in nearly all organized tissues. It is a member of the alkaline earth family of metals with the atomic symbol Ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes.Tyrosine 3-Monooxygenase: An enzyme that catalyzes the conversion of L-tyrosine, tetrahydrobiopterin, and oxygen to 3,4-dihydroxy-L-phenylalanine, dihydrobiopterin, and water. EC 18.104.22.168.Efferent Pathways: Nerve structures through which impulses are conducted from a nerve center toward a peripheral site. Such impulses are conducted via efferent neurons (NEURONS, EFFERENT), such as MOTOR NEURONS, autonomic neurons, and hypophyseal neurons.Extremities: The farthest or outermost projections of the body, such as the HAND and FOOT.Reflex: An involuntary movement or exercise of function in a part, excited in response to a stimulus applied to the periphery and transmitted to the brain or spinal cord.Neural Pathways: Neural tracts connecting one part of the nervous system with another.Macaca mulatta: A species of the genus MACACA inhabiting India, China, and other parts of Asia. The species is used extensively in biomedical research and adapts very well to living with humans.Neurotransmitter Agents: Substances used for their pharmacological actions on any aspect of neurotransmitter systems. Neurotransmitter agents include agonists, antagonists, degradation inhibitors, uptake inhibitors, depleters, precursors, and modulators of receptor function.LIM-Homeodomain Proteins: A subclass of LIM domain proteins that include an additional centrally-located homeodomain region that binds AT-rich sites on DNA. Many LIM-homeodomain proteins play a role as transcriptional regulators that direct cell fate.Caenorhabditis elegans Proteins: Proteins from the nematode species CAENORHABDITIS ELEGANS. The proteins from this species are the subject of scientific interest in the area of multicellular organism MORPHOGENESIS.Embryo, Mammalian: The entity of a developing mammal (MAMMALS), generally from the cleavage of a ZYGOTE to the end of embryonic differentiation of basic structures. For the human embryo, this represents the first two months of intrauterine development preceding the stages of the FETUS.Molecular Sequence Data: Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.Myenteric Plexus: One of two ganglionated neural networks which together form the ENTERIC NERVOUS SYSTEM. The myenteric (Auerbach's) plexus is located between the longitudinal and circular muscle layers of the gut. Its neurons project to the circular muscle, to other myenteric ganglia, to submucosal ganglia, or directly to the epithelium, and play an important role in regulating and patterning gut motility. (From FASEB J 1989;3:127-38)Movement Disorders: Syndromes which feature DYSKINESIAS as a cardinal manifestation of the disease process. Included in this category are degenerative, hereditary, post-infectious, medication-induced, post-inflammatory, and post-traumatic conditions.Brain Mapping: Imaging techniques used to colocalize sites of brain functions or physiological activity with brain structures.Thalamus: Paired bodies containing mostly GRAY MATTER and forming part of the lateral wall of the THIRD VENTRICLE of the brain.Hand: The distal part of the arm beyond the wrist in humans and primates, that includes the palm, fingers, and thumb.Brain-Derived Neurotrophic Factor: A member of the nerve growth factor family of trophic factors. In the brain BDNF has a trophic action on retinal, cholinergic, and dopaminergic neurons, and in the peripheral nervous system it acts on both motor and sensory neurons. (From Kendrew, The Encyclopedia of Molecular Biology, 1994)Pons: The front part of the hindbrain (RHOMBENCEPHALON) that lies between the MEDULLA and the midbrain (MESENCEPHALON) ventral to the cerebellum. It is composed of two parts, the dorsal and the ventral. The pons serves as a relay station for neural pathways between the CEREBELLUM to the CEREBRUM.Growth Cones: Bulbous enlargement of the growing tip of nerve axons and dendrites. They are crucial to neuronal development because of their pathfinding ability and their role in synaptogenesis.RNA-Binding Protein FUS: A multifunctional heterogeneous-nuclear ribonucleoprotein that may play a role in homologous DNA pairing and recombination. The N-terminal portion of protein is a potent transcriptional activator, while the C terminus is required for RNA binding. The name FUS refers to the fact that genetic recombination events result in fusion oncogene proteins (ONCOGENE PROTEINS, FUSION) that contain the N-terminal region of this protein. These fusion proteins have been found in myxoid liposarcoma (LIPOSARCOMA, MYXOID) and acute myeloid leukemia.Spinal Nerve Roots: Paired bundles of NERVE FIBERS entering and leaving the SPINAL CORD at each segment. The dorsal and ventral nerve roots join to form the mixed segmental spinal nerves. The dorsal roots are generally afferent, formed by the central projections of the spinal (dorsal root) ganglia sensory cells, and the ventral roots are efferent, comprising the axons of spinal motor and PREGANGLIONIC AUTONOMIC FIBERS.Substantia Nigra: The black substance in the ventral midbrain or the nucleus of cells containing the black substance. These cells produce DOPAMINE, an important neurotransmitter in regulation of the sensorimotor system and mood. The dark colored MELANIN is a by-product of dopamine synthesis.RNA, Messenger: RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.Proprioception: Sensory functions that transduce stimuli received by proprioceptive receptors in joints, tendons, muscles, and the INNER EAR into neural impulses to be transmitted to the CENTRAL NERVOUS SYSTEM. Proprioception provides sense of stationary positions and movements of one's body parts, and is important in maintaining KINESTHESIA and POSTURAL BALANCE.Receptors, N-Methyl-D-Aspartate: A class of ionotropic glutamate receptors characterized by affinity for N-methyl-D-aspartate. NMDA receptors have an allosteric binding site for glycine which must be occupied for the channel to open efficiently and a site within the channel itself to which magnesium ions bind in a voltage-dependent manner. The positive voltage dependence of channel conductance and the high permeability of the conducting channel to calcium ions (as well as to monovalent cations) are important in excitotoxicity and neuronal plasticity.Photic Stimulation: Investigative technique commonly used during ELECTROENCEPHALOGRAPHY in which a series of bright light flashes or visual patterns are used to elicit brain activity.Corpus Striatum: Striped GRAY MATTER and WHITE MATTER consisting of the NEOSTRIATUM and paleostriatum (GLOBUS PALLIDUS). It is located in front of and lateral to the THALAMUS in each cerebral hemisphere. The gray substance is made up of the CAUDATE NUCLEUS and the lentiform nucleus (the latter consisting of the GLOBUS PALLIDUS and PUTAMEN). The WHITE MATTER is the INTERNAL CAPSULE.Mechanoreceptors: Cells specialized to transduce mechanical stimuli and relay that information centrally in the nervous system. Mechanoreceptor cells include the INNER EAR hair cells, which mediate hearing and balance, and the various somatosensory receptors, often with non-neural accessory structures.Prosencephalon: The anterior of the three primitive cerebral vesicles of the embryonic brain arising from the NEURAL TUBE. It subdivides to form DIENCEPHALON and TELENCEPHALON. (Stedmans Medical Dictionary, 27th ed)Rotarod Performance Test: A performance test based on forced MOTOR ACTIVITY on a rotating rod, usually by a rodent. Parameters include the riding time (seconds) or endurance. Test is used to evaluate balance and coordination of the subjects, particular in experimental animal models for neurological disorders and drug effects.Neuroprotective Agents: Drugs intended to prevent damage to the brain or spinal cord from ischemia, stroke, convulsions, or trauma. Some must be administered before the event, but others may be effective for some time after. They act by a variety of mechanisms, but often directly or indirectly minimize the damage produced by endogenous excitatory amino acids.Phenotype: The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.Microelectrodes: Electrodes with an extremely small tip, used in a voltage clamp or other apparatus to stimulate or record bioelectric potentials of single cells intracellularly or extracellularly. (Dorland, 28th ed)Learning: Relatively permanent change in behavior that is the result of past experience or practice. The concept includes the acquisition of knowledge.Mice, Neurologic Mutants: Mice which carry mutant genes for neurologic defects or abnormalities.Excitatory Amino Acid Antagonists: Drugs that bind to but do not activate excitatory amino acid receptors, thereby blocking the actions of agonists.Gene Expression Regulation: Any of the processes by which nuclear, cytoplasmic, or intercellular factors influence the differential control (induction or repression) of gene action at the level of transcription or translation.Neurotoxins: Toxic substances from microorganisms, plants or animals that interfere with the functions of the nervous system. Most venoms contain neurotoxic substances. Myotoxins are included in this concept.Peripheral Nervous System: The nervous system outside of the brain and spinal cord. The peripheral nervous system has autonomic and somatic divisions. The autonomic nervous system includes the enteric, parasympathetic, and sympathetic subdivisions. The somatic nervous system includes the cranial and spinal nerves and their ganglia and the peripheral sensory receptors.Forelimb: A front limb of a quadruped. (The Random House College Dictionary, 1980)Transcription Factors: Endogenous substances, usually proteins, which are effective in the initiation, stimulation, or termination of the genetic transcription process.Nitrergic Neurons: Nerve cells where transmission is mediated by NITRIC OXIDE.Receptors, AMPA: A class of ionotropic glutamate receptors characterized by their affinity for the agonist AMPA (alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid).Nephropidae: Family of large marine CRUSTACEA, in the order DECAPODA. These are called clawed lobsters because they bear pincers on the first three pairs of legs. The American lobster and Cape lobster in the genus Homarus are commonly used for food.Parkinson Disease: A progressive, degenerative neurologic disease characterized by a TREMOR that is maximal at rest, retropulsion (i.e. a tendency to fall backwards), rigidity, stooped posture, slowness of voluntary movements, and a masklike facial expression. Pathologic features include loss of melanin containing neurons in the substantia nigra and other pigmented nuclei of the brainstem. LEWY BODIES are present in the substantia nigra and locus coeruleus but may also be found in a related condition (LEWY BODY DISEASE, DIFFUSE) characterized by dementia in combination with varying degrees of parkinsonism. (Adams et al., Principles of Neurology, 6th ed, p1059, pp1067-75)Stilbamidines: STILBENES with AMIDINES attached.Spinal Cord Injuries: Penetrating and non-penetrating injuries to the spinal cord resulting from traumatic external forces (e.g., WOUNDS, GUNSHOT; WHIPLASH INJURIES; etc.).Models, Biological: Theoretical representations that simulate the behavior or activity of biological processes or diseases. For disease models in living animals, DISEASE MODELS, ANIMAL is available. Biological models include the use of mathematical equations, computers, and other electronic equipment.Dose-Response Relationship, Drug: The relationship between the dose of an administered drug and the response of the organism to the drug.
Upper motor neuron syndrome. BTX-A is now a common treatment for muscles affected by the upper motor neuron syndrome ( ... Once bound to the nerve terminal, the neuron takes up the toxin into a vesicle by receptor-mediated endocytosis. As the ... the brain mechanism of motor fusion, which aligns the eyes on a target visible to both, can stabilize the corrected alignment.[ ... he concluded that the toxin acts by interrupting signal transmission in the somatic and autonomic motor systems, without ...
Upper motor neuron syndrome. BTX-A is now a common treatment for muscles affected by the upper motor neuron syndrome ( ... Once bound to the nerve terminal, the neuron takes up the toxin into a vesicle. As the vesicle moves farther into the cell, it ... If there is good binocular vision, the brain mechanism of motor fusion, which aligns the eyes on a target visible to both, can ... he concluded that the toxin acts by interrupting signal transmission in the somatic and autonomic motor systems, without ...
Stein, P., S. Grillner, A. Selverston, and D. Stuart (1997). Neurons, Networks, and Motor Behavior. MIT Press. ISBN 0-262-19390 ... including but not limited to cortical chattering neurons, thalamacortical neurons, and pacemaker neurons. Pacemakers in general ... "Neurons, Networks, and Motor Behavior." MIT Press (1997). ISBN 978-0-262-69227-4. ... Parabolic bursting has been studied most extensively in the R15 neuron, which is one of six types of neurons of the Aplysia ...
ISBN 978-0-306-31001-0. "Wormbook: Specification of the nervous system". Stein PSG (1999). Neurons, Networks, and Motor ... A command neuron is a special type of identified neuron, defined as a neuron that is capable of driving a specific behavior all ... A neuron is called identified if it has properties that distinguish it from every other neuron in the same animal-properties ... The concept of a command neuron has, however, become controversial, because of studies showing that some neurons that initially ...
Henneman's size principle
Motor neurons of different sizes have similar voltage thresholds. Smaller neurons have higher membrane resistance and require ... the physiological and anatomical features of oculomotor nucleus motor neurons remain unchanged. Rat oculomotor nucleus motor ... Henneman also looked at what order the motor neurons were inhibited as the stretch was released and found that it was the ... The increase in size of motor neurons led to a decrease in input resistance with a strong linear relationship in both age ...
Glossary of biology
monomer . motor neuron . mucous membrane . multicellular organism . muon The muon is an unstable subatomic particle with a mean ... DNA sequencing . dynein A motor protein (also called molecular motor or motor molecule) in cells which converts the chemical ... In a typical neuron, this is about -70 millivolts (mV). The minus sign indicates that the inside of the cell is negative with ... depolarization The process of reversing the charge across a cell membrane (usually a NEURON), so causing an ACTION POTENTIAL. ...
Primary lateral sclerosis
PLS belongs to a group of disorders known as motor neuron diseases. Motor neuron diseases develop when the nerve cells that ... PLS only affects upper motor neurons. There is no evidence of the degeneration of spinal motor neurons or muscle wasting ( ... Due to the fact that a person with ALS may initially present with only upper motor neuron symptoms, indicative of PLS, one key ... "Motor neuron diseases". Archived from the original on April 28, 2009. Retrieved 2009-06-02. Primary Lateral Sclerosis at ...
Motor Neuron Firing > Motor Output > Environmental Change > Sensory Transduction ... There are two main technical challenges ... sensory inputs and motor outputs. Inputs employed 16-bit A/D converters attached to operational amplifier simulated neurons and ... with its hermaphrodite type having only 302 neurons. Furthermore, the structural connectome of these neurons is fully worked ... With so few neurons and new calcium 2 photon microscopy techniques it should soon be possible to record the complete neural ...
Amyotrophic lateral sclerosis
In this case, neurons in the brain (upper motor neurons) and in the spinal cord (lower motor neurons) are dying and this form ... ALS is a motor neuron disease, also spelled "motor neurone disease", which is a group of neurological disorders that ... An abnormal reflex commonly called Babinski's sign also indicates upper motor neuron damage. Symptoms of lower motor neuron ... "Avoiding false positive diagnoses of motor neuron disease: lessons from the Scottish Motor Neuron Disease Register". J. Neurol ...
Hereditary spastic paraplegia
The affected cells are the primary motor neurons, therefore the disease is an upper motor neuron disease. HSP is not a form of ... HSP affects several pathways in motor neurons. Many genes were identified and linked to HSP. It remains a challenge to ... Dysfunction of endosomal trafficking can have severe consequences in motor neurons with long axons, as reported in HSP. ... If L1CAM does not work correctly due to a mutation, the cortiocospinal neurons are not directed to the correct position and the ...
S. Samar Hasnain
... what is unique about the AMPA receptors expressed on spinal motor neurons?". Amyotroph. Lateral Scler. Other Motor Neuron ... Q/R editing is not the only mechanism involved, as editing occurs only in spinal motor neurons not in upper spinal neurons. ... In diseased motor neurons editing levels of Glur 2 (62-100%) at this site was discovered to be reduced. Abnormal editing is ... With these conditions motor neurons degenerate leading to eventual paralysis and respiratory failure. Glutamate excitotoxicity ...
Epigenetics of neurodegenerative diseases
Neurodengenerative diseases of motor neurons can cause degeneration of motor neurons involved in voluntary muscle control such ... As SMN protein generally promotes the survival of motor neurons, mutations in SMN1 results in slow degeneration motor neurons ... is a motor neuron disease that involves neurogeneration. All skeletal muscles in the body are controlled by motor neurons that ... See the Motor Neuron Fact Sheet for details regarding other motor neuron diseases.Neurodegenerative diseases of the central ...
The motor information is sent through efferent neurons. In a doctor's office or in a research experiment, a person's perception ... and third order neurons. The first order neuron is the afferent neuron. The first order neuron enters the spinal cord through ... If the neurons are coming from the lower limbs are carried by the fasciculus gracilis into the medulla. If the neurons are ... Deterioration of the myelin sheath drastically reduces the conduction speed of neurons, thus affecting sensation and motor ...
Nuclear chain fiber
Tetanospasmin released in the wound is absorbed into the circulation and reaches the ends of motor neurons all over the body. ... By binding to peripheral motor neuron terminals, the toxin enters the nerve axons, and is transported across synaptic junctions ... These inhibitory neurotransmitters inhibit the alpha motor neurons. With diminished inhibition, the resting firing rate of the ... alpha motor neuron increases, producing rigidity, unopposed muscle contraction and spasm. Characteristic features are risus ...
GABA is a neurotransmitter that inhibits motor neurons. The action of the A-chain stops the affected neurons from releasing the ... "Myosin Va and microtubule-based motors are required for fast axonal retrograde transport of tetanus toxin in motor neurons". ... Once it is bound the neurotoxin is then endocytosed into the nerve and begins to travel through the axon to the spinal neurons ... The toxin bind to the neurons is irreversible and nerve function can only be returned by the growth of new terminals and ...
General somatic efferent fibers
Alpha motor neurons (α) target extrafusal muscle fibers. Gamma motor neurons (γ) target intrafusal muscle fibres Cranial nerves ... The *spinal* somatic efferent neurons (GSE, 'somatomotor, or somatic motor fibers), arise from motor neuron cell bodies in the ... They exit the spinal cord through the ventral roots, carrying motor impulses to skeletal muscle. Of the somatic efferent ... also supply their own somatic efferent neurons to the extraocular muscles and some of the muscles of the tongue. Nerve fiber ...
Christopher Shaw (neurologist)
His major research interest is in the genetic, molecular and cellular basis of motor neuron diseases such as amyotrophic ... "Breakthrough in the study of motor neurone disease". The Independent. Retrieved 8 April 2016. Vance, C.; Rogelj, B.; Hortobagyi ... "Motor neurone disease 'gene clue'". BBC. 28 February 2009. Retrieved 8 April 2016. ...
"Cloning and localization of a conventional kinesin motor expressed exclusively in neurons". Neuron. 12 (5): 1059-72. doi: ... a novel neuronal kinesin enriched in motor neurons". The Journal of Neuroscience. 20 (17): 6374-84. PMID 10964943. Setou M, ... Kull FJ, Sablin EP, Lau R, Fletterick RJ, Vale RD (Apr 1996). "Crystal structure of the kinesin motor domain reveals a ... Hakimi MA, Speicher DW, Shiekhattar R (Oct 2002). "The motor protein kinesin-1 links neurofibromin and merlin in a common ...
Wichterle H, Lieberam I, Porter JA, Jessell TM (2002). "Directed differentiation of embryonic stem cells into motor neurons". ... Stem cells are by definition pluripotent, able to differentiate into several cell types such as neurons, cardiomyocytes, ... cardiomyocytes and neurons. Drug screen are performed on miniaturized cell culture in multiwell-plates or on a chip. PSCs- ... "Retinoic acid induces embryonal carcinoma cells to differentiate into neurons and glial cells". The Journal of Cell Biology. ...
Emery N. Brown
ALS Functional Rating Scale - Revised
Beck Depression Inventory
... is a motor neuron disease that affects the brain and spinal cord. The disease causes motor neurons to degenerate, which ... "Gene Therapy for Motor Neuron Disease". Society for Neuroscience. Retrieved 13 December 2013. Ding H, Schwarz DS, Keene A, ... This disease is incurable and known to cause motor, cognitive, and behavioral deficits. Researchers have been looking to gene ... they found that the mice developed improved motor control and a longer survival rate when compared to the controls. Thus, gene ...
Single-neuron modeling. Main article: Biological neuron models. Even single neurons have complex biophysical ... doi:10.1016/j.neuron.2005.02.001. PMID 15721245.. *^ Coggan JS, Bartol TM, Esquenazi E, et al. (2005). "Evidence for ectopic ... The interactions of neurons in a small network can be often reduced to simple models such as the Ising model. The statistical ... Lapicque introduced the integrate and fire model of the neuron in a seminal article published in 1907. This model is still ...
In "kindling", repeated stimulation of hippocampal or amygdaloid neurons in the limbic system eventually leads to seizures in ... such as sensitization to the locomotor response of a stimulant resulting in cross-sensitization to the motor-activating effects ... In "central sensitization," nociceptive neurons in the dorsal horns of the spinal cord become sensitized by peripheral tissue ... A large body of literature has demonstrated that such ΔFosB induction in D1-type [nucleus accumbens] neurons increases an ...
This is due to the reduction of excitatory synaptic transmission in a nucleus and increased excitability in motor neurons ... There are two types of neurons in the pre-BötC: nonpacemaker and pacemaker neurons. Nonpacemaker neurons enter either a tonic ... intrinsically-bursting pacemaker neurons, and follower neurons within the pre-Bötzinger complex. Together these neurons make up ... Pacemaker neurons can further be subdivided into cadmium sensitive (CS) and cadmium insensitive (CI) pacemaker neurons. ...
Zullo, L.; Sumbre, G.; Agnisola, C.; Flash, T.; Hochner, B. (2009). "Nonsomatotopic organization of the higher motor centers in ... Two-thirds of an octopus's neurons are found in the nerve cords of its arms, which show a variety of complex reflex actions ... the proteins that guide the connections neurons make with each other. The California two-spot octopus has had its genome ... that persist even when they have no input from the brain. Unlike vertebrates, the complex motor skills of octopuses are not ...
Neo-Piagetian theories of cognitive development
അലൂമിനിയം ക്ലോറൈഡ് - വിക്കിപീഡിയ
"Neuron. 69 (3): 548-62. doi:10.1016/j.neuron.2010.11.045. PMC 3052770 . PMID 21315264.. ... The objection is that the time scales involved in motor control are very short, and motor control involves a great deal of ... the supplementary motor complex on the medial surface of the frontal lobe appears to activate prior to primary motor cortex ... David A. Rosenbaum (2009). Human Motor Control (2nd ed.). Academic Press. p. 86. ISBN 0123742269.. ...
Mirror neuron networks provide a mechanism for visuo-motor and motor-visual transformation and interaction. Similar networks of ... "The role of mirror neurons in observational motor learning: an integrative review". European Journal of Human Movement. 32: 82- ... These specialized visuomotor neurons fire action potentials when an individual performs a motor task and also fire when an ... In observational motor learning, the process begins with a visual presentation of another individual performing a motor task, ...
Infantile progressive bulbar palsy
The motor part of the spindle is provided by motor neurons: up to a dozen gamma motor neurons and one or two beta motor neurons ... Gamma motor neurons supply only muscle fibres within the spindle, whereas beta motor neurons supply muscle fibres both within ... muscle fibres within the spindle by up to a dozen gamma motor neurons and to a lesser extent by one or two beta motor neurons[ ... The function of the gamma motor neurons is not to supplement the force of muscle contraction provided by the extrafusal fibers ...
Brodmann area 45
doi:10.1016/j.neuron.2005.07.023.. *^ H. Jeong, M. Sugiura, Y. Sassa, T. Haji, N. Usui, M. Taira, K. Horie, S. Sato, R. ... 1999). "Effects of repetition and competition on activity of left prefrontal cortex during word generation". Neuron. 23 (3): ... Supplementary motor area *6. *Supplementary eye field *6. *Frontal eye fields *8 ...
Theodore Holmes Bullock
Neuroscience of music
Mirror/echo neurons and auditory-motor interactions. The mirror neuron system has an important role in neural models of ... The motor area processes the rhythm of the music (Dean, 2013). The motor area of the brain is located in the ... Auditory-motor interactions. Feedforward and feedback interactions. An auditory-motor interaction may be loosely ... It was concluded that a lesser amount of neurons needed to be activated for the piano players due to long-term motor practice ...
Hjernehinde, den frie encyklopædi
Hjernehinden (Skabelon:IPAc-en, singular: meninx (Skabelon:IPAc-en or Skabelon:IPAc-en), fra oldgræsk: μῆνιγξ,) er de tre membraner der slutter hjernen og rygmarven. Hos pattedyr er hjernehinderne dura mater, spindelhinden og pia mater. Rygmarvsvæske findes i cavum subarachnoideale mellem spindelhinden og pia mater. Hjernehindernes primære funktion er at beskytte centralnervesystemet. ...
Some of the branches of the I-a axons synapse directly with alpha motor neurons.These carry impulses back to the same muscle ... These, in turn, synapse with motor neurons leading back to the antagonistic muscle, a flexor in the back of the thigh. By ... where second-order neurons send the signal to the thalamus and synapse with third-order neurons in the ventrobasal complex. The ... Stretching a spindle fiber initiates a volley of impulses in the sensory neuron (a I-a neuron) attached to it. The impulses ...
Brain-derived neurotrophic factor
... motor neurons and skeletal muscle and it is also found in saliva. ... regulation of neuron differentiation. • neuron projection morphogenesis. • modulation of chemical synaptic transmission. • ... doi:10.1016/j.neuron.2011.02.034. PMID 21435558. S2CID 15373477.. *^ a b Matsuoka Y, Li X, Bennett V (June 2000). "Adducin: ... This is important as neuron morphology is critical in behavioral processes like learning and motor skills development. Research ...
নিতম্বাস্থি - উইকিপিডিয়া
Olfactory ensheathing cells
... myelinated axons of olfactory neurons in a similar way to which Schwann cells ensheath non-myelinated peripheral neurons. They ... Traumatic spinal cord damage causes a permanent loss of motor and sensory functions in the central nervous system, termed ... New olfactory receptor neurons must project their axons through the central nervous system to an olfactory bulb in order to be ... The mammalian olfactory system is unusual in that it has the ability to continuously regenerate its neurons during adulthood.[4 ...
Outline of brain mapping
The neuron doctrineEdit. *Neuron doctrine - A set of carefully constructed elementary set of observations regarding neurons. ... cognitive or motor) event stimuli. The voltage swing sequences are recorded and broken down by positive and negative, and by ... The Neuron doctrine postulates several elementary aspects of neurons: *The brain is made up of individual cells (neurons) that ... Neurons are generated by cell division.. *Neurons are connected by sites of contact and not via cytoplasmic continuity. (A cell ...
Various congenital and acquired tongue diseases can affect speech as can motor neuron disease. ... In speech repetition, speech being heard is quickly turned from sensory input into motor instructions needed for its immediate ... This is then sent from Broca's area to the motor cortex for articulation. ... where impaired motor planning, nerve transmission, phonological processing or perception of the message (as opposed to the ...
Amyotrophic lateral sclerosis
Typical or "classical" ALS involves neurons in the brain (upper motor neurons) and in the spinal cord (lower motor neurons).[27 ... Typical or "classical" ALS involves neurons in the brain (upper motor neurons) and in the spinal cord (lower motor neurons).[27 ... The defining feature of ALS is the death of both upper motor neurons (located in the motor cortex of the brain) and lower motor ... ALS is a motor neuron disease, also spelled "motor neurone disease", which is a group of neurological disorders that ...
Ikeda K, Akiyama H, Arai T, Ueno H, Tsuchiya K, Kosaka K (July 2002). "Morphometrical reappraisal of motor neuron system of ... and motor neuron diseases, polyglutamine (PolyQ) diseases, muscular dystrophies and several rare forms of ... primary cultures of quiescent and differentiated cells such as thymocytes and neurons - are prevented from undergoing apoptosis ...
Without efficient autophagy, neurons gather ubiquitinated protein aggregates and degrade. Ubiquitinated proteins are proteins ... The Dynamic Interaction of Ambra1 with the Dynein Motor Complex Regulates Mammalian Autophagy', J Cell Biol, 191 (2010), 155-68 ... Parkinson's disease is characterized by inclusions of a protein called alpha-synuclien (Lewy bodies) in affected neurons that ...
... from molecular and cellular studies of individual neurons to imaging of sensory and motor tasks in the brain. Neuroscience has ... Neurons are cells specialized for communication. They are able to communicate with neurons and other cell types through ... Llinas R. (2001). I of the Vortex: From Neurons to Self MIT Press. ISBN 0-262-12233-2 (Hardcover) ISBN 0-262-62163-0 (Paperback ... Many neurons extrude a long thin filament of axoplasm called an axon, which may extend to distant parts of the body and are ...
Without efficient autophagy, neurons gather ubiquitinated protein aggregates and degrade. Ubiquitinated proteins are proteins ... "The dynamic interaction of AMBRA1 with the dynein motor complex regulates mammalian autophagy". The Journal of Cell Biology ... Parkinson's disease is characterized by inclusions of a protein called alpha-synuclien (Lewy bodies) in affected neurons that ...
Upper motor neuron disease | pathology | Britannica.com
Progressive motor neuron disease legal definition of progressive motor neuron disease
What is progressive motor neuron disease? Meaning of progressive motor neuron disease as a legal term. What does progressive ... Definition of progressive motor neuron disease in the Legal Dictionary - by Free online English dictionary and encyclopedia. ... Progressive motor neuron disease legal definition of progressive motor neuron disease https://legal-dictionary. ... redirected from progressive motor neuron disease). Also found in: Dictionary, Thesaurus, Medical, Encyclopedia.. Related to ...
Motor Neurons News, Research
Ezogabine treatment reduces motor neuron excitability in ALS patients, study shows Brian Wainger, MD, PhD, of the Healey Center ... Amyotrophic lateral sclerosis is a motor neuron disease with rampant progression and that it is typically fatal within 5 years ... The neurodegenerative disease ALS causes motor neuron death and paralysis. However, long before the cells die, they lose ... Virginia Tech Carilion Research Institute scientists have shown that mutations in specific genes that destroy motor neurons and ...
motor neuron disease - Wiktionary
Neurons, Networks, and Motor Behavior | The MIT Press
At the cellular level, Neurons, Networks, and Motor Behavior describes the computational characteristics of individual neurons ... Recent advances in motor behavior research rely on detailed knowledge of the characteristics of the neurons and networks that ... Home Computer Science and Intelligent Systems Neuroscience Neurons, Networks, and Motor Behavior ... selection and initiation of motor patterns; generation and formation of motor patterns: cellular and systems properties; ...
Motor neurone disease. | The BMJ
Motor neuron - Latest research and news | Nature
Motor neuron. Definition. Motor neurons project from the central nervous system to innervate muscle cells to either directly or ... The subtype of motor neurons that is most likely to degenerate early in amyotrophic lateral sclerosis is prone to endoplasmic ... Motor neuron development in zebrafish is altered by brief (5-hr) exposures to THC (∆9-tetrahydrocannabinol) or CBD (cannabidiol ... Contact between a motor neuron and muscle cell is usually via a specialized synapse called a neuromuscular junction. ...
Grafts of embryonic neurons restore damaged motor pathways | ScienceBlogs
French researchers have demonstrated for the first time that embryonic cells grafted into the brains of mice with damaged motor ... 2007). Reestablishment of damaged adult motor pathways by grafted embryonic cortical neurons. Nat. Neurosci. doi: 10.1038/ ... Immediately afterwards, motor cortical tissue from donor embryos was transplanted into the lesioned site. Care was taken to ... Gaillard, et al used donor tissue came from transgenic mice that express green fluorescent protein in all neurons. The ...
Epidemiology of Motor Neuron Disease | SpringerLink
Kondo K. (1995) Epidemiology of Motor Neuron Disease. In: Leigh P.N., Swash M. (eds) Motor Neuron Disease. Springer, London. * ... Amyotrophic Lateral Sclerosis Motor Neuron Standardise Mortality Ratio Motor Neuron Disease Mariana Island These keywords were ... Kondo K (1987) Environmental factors in motor neurone disease. In: Gourie-Devi M (ed) Motor neurone disease. Oxford University ... Epidemiology of motor neurone disease; ageing and exhaustion hypotheses revisited. In: Rose FC (ed) Research progress in motor ...
Motor Neurone Disease Treatment
There is currently no known cure for motor neurone disease and treatment is aimed at easing symptoms and helping patients to ... www.nhs.uk/conditions/Motor-neurone-disease/Pages/Introduction.aspx. *www.neura.edu.au/.../...%20the%20patient%20in%20general% ... Motor Neurone Disease Treatment. News-Medical. 17 October 2019. ,https://www.news-medical.net/health/Motor-Neurone-Disease- ... Motor Neurone Disease Treatment. News-Medical, viewed 17 October 2019, https://www.news-medical.net/health/Motor-Neurone- ...
BBC NEWS | Health | Motor neurone disease clue found
Scientists have identified a molecule which could be key to understanding the cause of motor neurone disease. ... Scientists have identified a molecule which could be key to understanding the cause of motor neurone disease (MND) and other ... Dr Belinda Cupid, of the MND Association, said: "We know from recent research that signs of motor neurone damage, on a cellular ... level, in models of MND occur very much earlier than the symptoms appear, so any new knowledge of how healthy motor neurones ...
What is the anatomy of motor neurons in diabetic neuropathy?
Motor neurons originate in the central nervous system (CNS) and extend to the anterior horn of the spinal cord. From the ... encoded search term (What is the anatomy of motor neurons in diabetic neuropathy?) and What is the anatomy of motor neurons in ... Motor neurons originate in the central nervous system (CNS) and extend to the anterior horn of the spinal cord. From the ... What is the anatomy of motor neurons in diabetic neuropathy?. Updated: Jan 17, 2020 ...
Study investigates how to turn stem cells into motor neurons
A team of researchers investigates details of the cellular mechanisms involved in transforming a stem cell into a motor neuron ... A team of researchers uncovers new details involved in the process of turning stem cells into motor neurons. New research ... "We have a very efficient system in which we can transform stem cells into motor neurons with something like a 90 to 95 percent ... "Study investigates how to turn stem cells into motor neurons." Medical News Today. MediLexicon, Intl., 9 Dec. 2016. Web.. 25 ...
Understanding Motor Neurone Disease - Afternoons - ABC Radio
Motor Neurone Disease Association of NSW - Idealist
... to provide the best possible assistance and information for people living with motor neurone disease, those whose diagnosis is ... to provide the best possible assistance and information for people living with motor neurone disease, those whose diagnosis is ... Motor Neurone Disease Association of NSW. NSW, Australia. , http://www.mndnsw.asn.au ...
Upper motor neurons (video) | Khan Academy
So upper motor neurons, which are different than the motor neurons we talked about before, which are the lower motor neurons. ... And it turns out there are upper motor neuron signs and just like with the lower motor neuron signs the upper motor neuron ... apparently without periodic stimulation of the lower motor neurons from the upper motor neurons, the lower motor neurons may ... The upper motor neuron thats going to control this lower motor neuron is going to start somewhere way up in the cerebral ...
motor neurone disease
... Lyn Frumkin lrfrum at hardy.u.washington.edu Sun Jul 16 06:08:37 EST 1995 *Previous message: motor ... My father in law has motor neurone disease, as did my own father. : ,Any one know of the latest in research in this topic with ... Will post responses also to a specific motor-neurone group which has : ,very little traffic. : , : ,Dr.Alex Alexander MBBS PhD( ... Alexander, : I dont know what you mean by motor neurone disease (peripheral, central, MD, : MS, etc.?), but as a Ph. D. ...
New brain research reveals that motor neurons adjust to control tasks
... Published Thursday 20 April 2017 Published Thu 20 Apr ... 2017, April 20). "New brain research reveals that motor neurons adjust to control tasks." Medical News Today. Retrieved from. ... "New brain research reveals that motor neurons adjust to control tasks." Medical News Today. MediLexicon, Intl., 20 Apr. 2017. ... The results revealed that dynamic range adaptation did indeed occur in the motor cortical neurons. Based on these findings, the ...
Specialized coding of sensory, motor and cognitive variables in VTA dopamine neurons | Nature
As mice navigated in a virtual-reality environment, dopamine neurons encoded an array of sensory, motor and cognitive variables ... Whether individual dopamine neurons multiplex several variables, or whether there are subsets of neurons that are specialized ... Two-photon calcium imaging of a large population of dopamine neurons in the ventral tegmental area of mice performing a virtual ... An important question is how the responses to these diverse variables are organized across the population of dopamine neurons. ...
Motor neurone disease: a demeaning illness. | The BMJ
Motor Neuron Disease | The Irish Times
Multidisciplinary Interventions in Motor Neuron Disease
O. Hardiman, "Multidisciplinary care in motor neurone disease," in The Motor Neurone Disease Handbook, M. Kiernan, Ed., ... "Correlates of quality of life in people with motor neuron disease (MND)," Amyotrophic Lateral Sclerosis and Other Motor Neuron ... Multidisciplinary Interventions in Motor Neuron Disease. U. E. Williams, E. E. Philip-Ephraim, and S. K. Oparah ... M. R. Turner and A. Al-Chalabi, "Clinical phenotypes," in The Motor Neurone Disease Handbook, M. Kiernan, Ed., pp. 55-73, ...
Ekklesia | Motor Neurone Disease
Research by the Motor Neurone Disease Association has found that a third of unpaid carers spend more than 100 hours a week ... The Motor Neurone Disease Association says the governments new strategy fails to acknowledge that employment-related ... There can be few things more devastating than to be diagnosed with Motor Neurone Disease (MND ... revealing terminally ill people face devastating and far-reaching financial hardship has been welcomed by the Motor Neurone ...
Distinct subclasses of medium spiny neurons differentially regulate striatal motor behaviors | PNAS
Distinct subclasses of medium spiny neurons differentially regulate striatal motor behaviors. Helen S. Bateup, Emanuela Santini ... Distinct subclasses of medium spiny neurons differentially regulate striatal motor behaviors. Helen S. Bateup, Emanuela Santini ... Distinct subclasses of medium spiny neurons differentially regulate striatal motor behaviors Message Subject (Your Name) has ... Distinct subclasses of medium spiny neurons differentially regulate striatal motor behaviors. Helen S. Bateup, Emanuela Santini ...
Trophic protection of motor neurons: clinical potential in motor neuron diseases | SpringerLink
... on motor neuron dysfunction in wobbler mouse motor neuron disease. Ann. Neurol (in press)Google Scholar ... Neurodegenerative Disease Motor Neuron Nerve Growth Factor Neurotrophic Factor Sensory Neuron These keywords were added by ... on motor dysfunction in wobbler mouse motor neuron disease. Ann. Neurol (submitted)Google Scholar ... Ciliary neurotrophic factor prevents degeneration of motor neurons in mouse mutant progressive motor neuronopathy. Nature 358: ...
Megan Taylor is fundraising for Motor Neurone Disease Association
King's College London - Genetic testing for motor neurone disease
... the most common form of motor neuron disease. In ALS, motor neurons in the brain and spinal cord degenerate, causing the ... The team has also demonstrated, in ALS motor neurons (pictured above), that mutated TDP-43 protein accumulates inside motor ... Genetic testing for motor neurone disease. Our researchers developed diagnostic tests that led to the first child being born ... Home , Institute of Psychiatry, Psychology & Neuroscience , About , Genetic testing for motor neurone disease ...
Motor Neuron Disease (MND): 7 Types, Causes, Symptoms & Treatment
... is the most common type of motor neuron disease. WebMD explains the other types and how they can affect your muscles. ... What Are Motor Neuron Diseases?. What Are Motor Neuron Diseases? What Are Motor Neuron Diseases? * What Are Motor Neurons? ... Living With a Motor Neuron Disease When you take a walk, talk to a friend, or chew a piece of food, motor neurons are behind ... Living With a Motor Neuron Disease. The outlook is different for each type of motor neuron disease. Some are milder and ...
Mitochondrial Chromosome Disintegrates in ALS Motor Neurons | ALZFORUM
In the first study to count mitochondrial genes in single motor neurons, researchers have found that motor neurons from people ... This was common in ALS spinal neurons, Bennett said, and slightly less common in control motor neurons. However, not all ALS ... Keeney and Bennett also examined copy number in the Purkinje neurons and found it less variable than in motor neurons, ... Motor neurons from both ALS and control samples had widely varying copy numbers, ranging from zero to 300,000. This kind of ...
Brain's tiniest blood vessels trigger spinal motor neurons to develop | EurekAlert! Science News
To make the discovery, investigators successfully re-created living tissues of the blood vessels and the spinal motor neurons ... prompting the neurons to grow during early development. The findings could provide insights into how amyotrophic lateral ... A new study has revealed that the human brains tiniest blood vessels can activate genes known to trigger spinal motor neurons ... "What may go wrong in the spinal neurons that causes the motor neurons to die?" Sances asked. "If we can model an individual ALS ...
Temporal Encoding of Movement in Motor Cortical Neurons | Journal of Neuroscience
Temporal Encoding of Movement in Motor Cortical Neurons. J. Andrew Pruszynski, Angela M. Coderre, Timothy P. Lillicrap, Isaac ... Temporal Encoding of Movement in Motor Cortical Neurons. J. Andrew Pruszynski, Angela M. Coderre, Timothy P. Lillicrap, Isaac ... Temporal Encoding of Movement in Motor Cortical Neurons Message Subject (Your Name) has forwarded a page to you from Journal of ... Temporal Encoding of Movement in Motor Cortical Neurons. J. Andrew Pruszynski, Angela M. Coderre, Timothy P. Lillicrap and ...
DiseaseAmyotrophicDisease AssociationAlpha motor nEmbryonicPluripotent stemCortical neuronsSensory neurons2019Upper motor neuron syTypes of motor neuronResearchersIndividual spinal motor neuronsDeath of motor neuronsAspects of motor neuronClinicalDisordersAxonsNeurologySomaticNerve cellsNeurologicalPhysiologyTargetsPathwaySubtypesSpinal neuronsDopamine neuronsDopaminergic neuronsSporadicMiceFrontotemporal dementiaPopulationsNervous SystemGenesControlSyndromeEpidemiologyStem cellsNeuronesMovementGeneNerves
- Amyotrophic lateral sclerosis is a motor neuron disease with rampant progression and that it is typically fatal within 5 years of prognosis. (news-medical.net)
- The subtype of motor neurons that is most likely to degenerate early in amyotrophic lateral sclerosis is prone to endoplasmic reticulum (ER) stress in mice, owing to low levels of SIL1, an ER-associated protein. (nature.com)
- A team of researchers from Harvard and Columbia University Medical Center have reprogrammed skin cells from an 82-year-old woman suffering from amyotrophic lateral sclerosis to generate first stem cells and then motor neurons. (scienceblogs.com)
- aray at emory.ed The term 'motor neuron disease' is used synonymous with the disease amyotrophic lateral sclerosis [also called Lou Gehrig's disease or ALS]. (bio.net)
- Every day, almost 400 people around the world are diagnosed with amyotrophic lateral sclerosis (ALS), the most common form of motor neurone disease. (kcl.ac.uk)
- In the first study to count mitochondrial genes in single motor neurons, researchers have found that motor neurons from people who had died of amyotrophic lateral sclerosis are likely to have mitochondrial DNA deficiencies and deletions. (alzforum.org)
- People with the commonest form of motor neuron disease (MND) called amyotrophic lateral sclerosis (ALS) are more likely to have relatively long ring fingers, reveals research from the Institute of Psychiatry (IoP) at King's. (kcl.ac.uk)
- This discovery, published in Nature Biotechnology , will make it possible to expand the production process for these neurons, leading to more rapid progress in understanding diseases of the motor system, such as infantile spinal amyotrophy or amyotrophic lateral sclerosis (ALS). (eurekalert.org)
- Motor neuron diseases are typically referred to as amyotrophic lateral sclerosis . (wisegeek.com)
- A Northwestern Medicine researcher has managed to isolate the elusive motor neurons in the brain's cortex that play an important role in the development of ALS (amyotrophic lateral sclerosis) and has managed to dress them in a green fluorescent color through which they can be easily identified and studied. (medindia.net)
- Damage of these neurons can cause very diverse diseases, for example spinal muscular atrophy in children or adult amyotrophic lateral sclerosis. (medicalxpress.com)
- Motor neurone disease (sometimes called Lou Gehrig 's disease or Amyotrophic lateral sclerosis ) is a chronic , progressive, almost always fatal neurological disease . (wikipedia.org)
- Amyotrophic lateral sclerosis (ALS) is the most common of the five types of motor neuron disease. (wikipedia.org)
- van der Graaff MM, de Jong JM, Baas F, de Visser M. Upper motor neuron and extra-motor neuron involvement in amyotrophic lateral sclerosis: a clinical and brain imaging review. (medscape.com)
- Intrinsic membrane hyperexcitability of amyotrophic lateral sclerosis patient-derived motor neurons. (broadinstitute.org)
- Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease of the motor nervous system. (broadinstitute.org)
- Facial onset sensory motor neuronopathy (FOSMN) syndrome: an unusual amyotrophic lateral sclerosis phenotype? (bmj.com)
- Optinurin inclusions in proximal hereditary motor and sensory neuropathy (HMSN-P): familial amyotrophic lateral sclerosis with sensory neuronopathy? (bmj.com)
- The aim of this study is to survey the effect of Tamoxifen in motor neuron disease (MND) patients, amyotrophic lateral sclerosis ( ALS ) with regular riluzole usage. (clinicaltrials.gov)
- A report revealing terminally ill people face devastating and far-reaching financial hardship has been welcomed by the Motor Neurone Disease Association. (ekklesia.co.uk)
- Research by the Motor Neurone Disease Association has found that a third of unpaid carers spend more than 100 hours a week caring and nearly half do not receive benefits to help support them. (ekklesia.co.uk)
- The Motor Neurone Disease Association says the government's new strategy fails to acknowledge that employment-related initiatives are not suitable for all disabled people. (ekklesia.co.uk)
- Our clinic is supported by the Motor Neuron Disease Association (MNDA) and the essence of our service is to provide a relaxed and calm environment to enable prolonged, careful and frank discussion. (uclh.nhs.uk)
- The Motor Neurone Disease Association has appointed Spencer du Bois to review its brand positioning. (designweek.co.uk)
- The Motor Neurone DIsease Association told Parliament's Work and Pensions Conmmittee, "Although Motor Neurone Disease (MND) is a terminal illness, and the Special Rules system nominally exists for those who are terminally ill, it does not work reliably for people with MND. (ekklesia.co.uk)
- The Motor Neurone Disease Association of Queensland Inc. receive limited recurrent funding and rely heavily on small grants and community support to enable us to continue providing the following services. (givenow.com.au)
- Motor Neurone Disease Association of SA (MND SA) provides client support services to all people in South Australia whose lives have been affected by MND. (givenow.com.au)
Alpha motor n10
- Alpha motor neurons are distinct from gamma motor neurons , which innervate intrafusal muscle fibers of muscle spindles . (wikipedia.org)
- Alpha motor neurons are derived from the basal plate (basal lamina) of the developing embryo . (wikipedia.org)
- A motor neuron pool contains the cell bodies of all the alpha motor neurons involved in contracting a single muscle. (wikipedia.org)
- Alpha motor neurons are located in lamina IX according to the Rexed lamina system . (wikipedia.org)
- Alpha motor neurons are located in a specific region of the spinal cord's gray matter. (wikipedia.org)
- Motor neuron is often associated with efferent neuron, primary neuron, or alpha motor neurons. (medicalxpress.com)
- Types of lower motor neurons are alpha motor neurons, beta motor neurons, and gamma motor neurons. (wikipedia.org)
- Alpha motor neurons innervate extrafusal muscle fibers, which are the main force-generating component of a muscle. (wikipedia.org)
- Alpha motor neurons are responsible for skeletal muscle contractions and movement. (alleydog.com)
- When the alpha motor neurons are activated the skeletal muscle fibers contract. (alleydog.com)
- French researchers have demonstrated for the first time that embryonic cells grafted into the brains of mice with damaged motor cortices can re-establish damaged connections precisely, so that disrupted neural circuitry is reconstructed. (scienceblogs.com)
- 2007). Reestablishment of damaged adult motor pathways by grafted embryonic cortical neurons. (scienceblogs.com)
- Henderson CE, Camu W, Mettling C, et al (1993) Neurotrophins promote motor neuron survival and are present in embryonic limb bud. (springer.com)
- The researchers cultured neurons from embryonic rat spinal cords and expressed the gene for human superoxide dismutase 1 (SOD1) in the cells. (alzforum.org)
- We have developed a series of mouse-mouse neural hybrid cell lines by fusing the aminopterin-sensitive neuroblastoma N18TG2 with motor neuron-enriched embryonic day 12-14 spinal cord cells. (nih.gov)
- Although scientists can flag spinal cord motor neurons in fluorescence, it wears off as the neuron ages because the process uses an embryonic gene. (medindia.net)
- Motor neurons begin to develop early in embryonic development, and motor function continues to develop well into childhood. (wikipedia.org)
- Human embryonic stem cell-derived motor neurons are sensitive to the toxic effect of glial cells carrying an ALS-causing mutation. (als.net)
- A method of differentiating embryonic stem cells into neural and motor cells is disclosed. (google.es)
- Soft culture substrates improve the yield of functional motor neurons derived from human pluripotent stem cells. (nature.com)
- Cedars-Sinai investigators first took samples of skin cells from adults and genetically reprogramed them into induced pluripotent stem cells, which can create any type of cell--in this case, spinal motor neurons and the lining of the brain's capillaries. (eurekalert.org)
- However, the development and realisation of these clinical applications is often limited by the inability to obtain specialised cells such as motor neurons from human pluripotent stem cells in an efficient and targeted manner. (eurekalert.org)
- 2008) Induced pluripotent stem cells generated from patients with ALS can be differentiated into motor neurons. (als.net)
- New research from Carnegie Mellon University's College of Engineering and the University of Pittsburgh reveals that motor cortical neurons optimally adjust how they encode movements in a task-specific manner. (medicalnewstoday.com)
- The study explored the change in brain activity during simple motor tasks performed through virtual reality in both 2-D and 3-D. The researchers wanted to know if the motor cortical neurons would automatically adjust their sensitivity to direction when presented with a wide range of possible directions instead of a narrow one. (medicalnewstoday.com)
- The results revealed that dynamic range adaptation did indeed occur in the motor cortical neurons. (medicalnewstoday.com)
- PITTSBURGH, April 18, 2017 - New research from Carnegie Mellon University's College of Engineering and the University of Pittsburgh reveals that motor cortical neurons optimally adjust how they encode movements in a task-specific manner. (upmc.com)
- First characterized as a target-derived survival factor for developing sympathetic and sensory neurons, it is now clear that NGF plays an important role in the maintenance and regeneration of mature peripheral neurons. (springer.com)
- The highly restricted specificity of NGF for sympathetic neurons, sub-populations of neural crest-derived sensory neurons and striatal and basal forebrain cholinergic neurons has for almost two decades spurred the search for other neurotrophic factors with specificities directed to the many classes of neurons which do not respond to NGF. (springer.com)
- The assembly of circuits within the spinal cord requires the generation of diverse cell types to accommodate the intricate sets of interconnections between motor neurons, sensory neurons, interneurons, and muscle. (nih.gov)
- All of the subsequent steps in motor neuron connectivity, such as their descending inputs from higher brain centers, their circuits with sensory neurons and interneurons are constrained by the early connections formed between motor neurons and their muscle targets. (nih.gov)
Upper motor neuron sy5
- The Upper Limb Reconstruction in Upper Motor Neuron Syndrome Clinic offers specialty care for people who've suffered an upper motor neuron (UMN) injury within their central nervous system and who now have shoulder, elbow, wrist, or hand dysfunction or deformity. (mayoclinic.org)
- An integrated team of physicians, diagnosticians and surgeons at Mayo Clinic's Rochester, Minnesota, campus brings extensive experience and specialized skills to your personalized care in a compassionate, supportive environment, offering you the most effective options for diagnosis and treatment of upper motor neuron syndrome. (mayoclinic.org)
- What is upper motor neuron syndrome? (mayoclinic.org)
- Upper motor neuron syndrome refers to a combination of resulting symptoms such as muscle weakness, decreased muscle control, easy fatigability, altered muscle tone and exaggerated deep tendon reflexes (also known as spasticity ), all of which can occur after a brain or spinal cord injury. (mayoclinic.org)
- The resulting changes in muscle performance that can be wide and varied are described overall as upper motor neuron syndrome. (wikipedia.org)
Types of motor neuron1
- Researchers at Karolinska Institutet in Sweden have developed a stem cell-based model in order to study the resilience and vulnerability of neurons in the neurodegenerative disease ALS. (news-medical.net)
- Optogenetics is a recently developed technique based on microbial proteins called channelrhodopsins (ChRs), which render neurons sensitive to light when inserted into them, thus enabling researchers to manipulate the activity of the cells using laser pulses. (scienceblogs.com)
- A team of researchers uncovers new details involved in the process of turning stem cells into motor neurons. (medicalnewstoday.com)
- Previous research in the field has suggested that this phenomenon, called dynamic range adaptation, is known to occur in neurons sensitive to sound, touch, and light - prompting the researchers to ask if the same phenomena would apply to neurons in the motor system that are associated with movement. (medicalnewstoday.com)
- An international study involving Irish researchers has identified a gene associated with Motor Neurone Disease. (irishtimes.com)
- Our researchers developed diagnostic tests that led to the first child being born free of the genetic mutation which causes motor neurone disease. (kcl.ac.uk)
- First author Paula Keeney of the University of Virginia in Charlottesville used laser capture microdissection to collect individual spinal motor neurons, then the researchers analyzed their mtDNA content. (alzforum.org)
- The researchers have shown that mitochondria are likely to malfunction in anterior spinal motor neurons, which could account for the mitochondrial problems in spinal cord homogenates. (alzforum.org)
- In the January 18 Journal of Neuroscience, researchers from Children's Hospital of Philadelphia, Pennsylvania, describe how blocking AMP activated protein kinase-normally a beneficial modulator of cellular metabolism-protects motor neurons in cell and animal models of the disease. (alzforum.org)
- Researchers are testing whether different drugs, agents, or interventions are safe and effective in slowing the progression of motor neuron diseasess. (nih.gov)
- This is the first time that researchers have been able to recreate apparently functional motor neurons in the laboratory from a person with ALS. (als.net)
- Researchers discovered that induced ALS motor neurons accumulate misfolded TDP-43 (green). (als.net)
- Peering under the microscope, the researchers found that the resulting motor neurons somewhat resembled those in people with ALS. (als.net)
- Two-photon imaging let researchers see how different types of motor neurons in the spinal cord alternate activity (shown by bright green fluorescence here) in a cyclical pattern. (bioopticsworld.com)
Individual spinal motor neurons1
Death of motor neurons2
- Rapid access to large quantities of neurons will be useful for testing a significant number of pharmacological drugs in order to identify those capable of preventing the death of motor neurons," concludes Cécile Martinat. (eurekalert.org)
- Most of ALS research has focused on the death of motor neurons in the spinal cord. (medindia.net)
Aspects of motor neuron2
- This is an exciting result that will motivate further research into motor learning and future clinical applications. (medicalnewstoday.com)
- The clinical nurse specialist and the Motor Neuron Disease (MND) care centre coordinator play key roles in providing information, advice and support to patients, their families and friends and to assist in the coordination of services. (uclh.nhs.uk)
- We show using multielectrode array and patch-clamp recordings that hyperexcitability detected by clinical neurophysiological studies of ALS patients is recapitulated in induced pluripotent stem cell-derived motor neurons from ALS patients harboring superoxide dismutase 1 (SOD1), C9orf72, and fused-in-sarcoma mutations. (broadinstitute.org)
- Scientists have identified a molecule which could be key to understanding the cause of motor neurone disease (MND) and other neurodegenerative disorders. (bbc.co.uk)
- At the National Hospital for Neurology and Neurosurgery at Queen Square we see a wide variety of rare neurological disorders and not everyone referred to this clinic has Motor Neuron Disease. (uclh.nhs.uk)
- The National Institute of Neurological Disorders and Stroke (NINDS) conducts and funds research on the motor neuron disorders. (nih.gov)
- In zebrafish, components of the extracellular matrix help to direct regenerating motor axons to their original targets. (nature.com)
- The somas of the upper motor neurons are found mainly up in the cerebral cortex, way up here in the brain on that outside surface of the cerebrum, and their axons descend down to synapse on lower motor neurons in the brain stem or in the spinal cord. (khanacademy.org)
- So first let's look at these longer upper motor neurons that send axons all the way down into the spinal cord. (khanacademy.org)
- So this pathway, this collection of axons that are upper motor neurons, traveling from the cerebral cortex to lower motor neurons in the spinal cord, we call this the corticospinal tract. (khanacademy.org)
- How motor neuron subclasses develop and extend axons to their correct targets is still poorly understood. (nih.gov)
- We show that LIM homeodomain factors Lhx3 and Lhx4 are expressed transiently in motor neurons whose axons emerge ventrally from the neural tube (v-MN). (nih.gov)
- Motor neurons develop in embryos deficient in both Lhx3 and Lhx4, but v-MN cells switch their subclass identity to become motor neurons that extend axons dorsally from the neural tube (d-MN). (nih.gov)
- Thus, Lhx3 and Lhx4 act in a binary fashion during a brief period in development to specify the trajectory of motor axons from the neural tube. (nih.gov)
- Axons from upper motor neurons synapse onto interneurons in the spinal cord and occasionally directly onto lower motor neurons. (wikipedia.org)
- The axons of motor neurons begin to appear in the fourth week of development from the ventral region of the ventral-dorsal axis (the basal plate). (wikipedia.org)
- Just before they enter the spinal cord, the spinal roots divide into the dorsal (posterior) and ventral (anterior) roots that respectively hold sensory and motor axons (nerve fibers). (rutgers.edu)
- Low index-to-ring finger length ratio in sporadic ALS supports prenatally defined motor neuronal vulnerability' is published in the Journal of Neurology, Neurosurgery and Psychiatry . (kcl.ac.uk)
- A new toxic entity associated with genetically inherited forms of dementia and motor neuron disease has been identified by scientists at the UCL Institute of Neurology. (redorbit.com)
- Somatic Motoneurons and Descending Motor Pathways. (springer.com)
- The target of these neurons varies, but in the somatic nervous system the target will be some sort of muscle fiber. (wikipedia.org)
- Units deprived of their adequate somatic, vestibular, and auditory stimuli showed undiminished discharge rates during motor activity. (sciencemag.org)
- All nerve cells communicate using special chemical messengers called neurotransmitters and people with motor neurone disease have an increased sensitivity to the neurotransmitter glutamate. (news-medical.net)
- Each kind of motor neuron disease affects different types of nerve cells or has a different cause. (webmd.com)
- ALS, the focus of the worldwide Ice Bucket Challenge in 2014, which raised a staggering £88 million or $115 million, is a progressive degenerative disease affecting nerve cells in the brain and spinal cord (motor neurons). (thesun.co.uk)
- Also known as ALS, the disease is a progressive degenerative disease affecting nerve cells in the brain and spinal cord (motor neurons). (thesun.co.uk)
- ALS, also known as Lou Gehrig's disease, causes the death of muscle-controlling nerve cells in the brain and spinal cord (motor neurons). (medindia.net)
- Specifically, nerve cells known as motor neurones are affected by MND. (hse.ie)
- Each neuron also has one process called an axon, which passes information to other nerve cells, or to effector cells such as muscle fibres. (sciencephoto.com)
- Motor Neuron disease is a neurological disease that destroys motor neurons, the cells that control essential voluntary muscle activity such as speaking, walking, breathing, and swallowing. (mycause.com.au)
- Motor Neurone Disease (MND) is a neurological disease that affects over 350,000 of the world's population, and kills over 100,000 every year. (mycause.com.au)
- Analysis of the distinct contributions of each pathway to behavior has been a challenge, however, due to the difficulty of selectively investigating the neurons comprising the two pathways using conventional techniques. (pnas.org)
- Direct confirmation of these hypotheses has been hindered by difficulty in selectively targeting direct and indirect pathway neurons with traditional surgical and pharmacological techniques. (pnas.org)
- To gain a better understanding of how the two output pathways regulate striatal motor responses, we selectively deleted the central signaling protein DARPP-32 in direct pathway striatonigral neurons or indirect pathway striatopallidal neurons. (pnas.org)
- This quality standard has been incorporated into the NICE pathway on motor neurone disease . (nice.org.uk)
- In this interactive object, learners examine the neuron pathway into and out of the spinal cord. (wisc-online.com)
- Autophagy pathway can be activated by mTOR inhibition, resulting in ameliorating TDP-43 accumulation and rescue in motor function in animal model. (clinicaltrials.gov)
- An important question is how the responses to these diverse variables are organized across the population of dopamine neurons. (nature.com)
- Whether individual dopamine neurons multiplex several variables, or whether there are subsets of neurons that are specialized in encoding specific behavioural variables remains unclear. (nature.com)
- This fundamental question has been difficult to resolve because recordings from large populations of individual dopamine neurons have not been performed in a behavioural task with sufficient complexity to examine these diverse variables simultaneously. (nature.com)
- Here, to address this gap, we used two-photon calcium imaging through an implanted lens to record the activity of more than 300 dopamine neurons from the ventral tegmental area of the mouse midbrain during a complex decision-making task. (nature.com)
- As mice navigated in a virtual-reality environment, dopamine neurons encoded an array of sensory, motor and cognitive variables. (nature.com)
- Together with the topography between dopamine neurons and their projections, this specialization and anatomical organization may aid downstream circuits in correctly interpreting the wide range of signals transmitted by dopamine neurons. (nature.com)
- Coddington, L. T. & Dudman, J. T. The timing of action determines reward prediction signals in identified midbrain dopamine neurons. (nature.com)
- Gaillard, et al used donor tissue came from transgenic mice that express green fluorescent protein in all neurons. (scienceblogs.com)
- The French team anaesthetized adult mice, and removed their motor cortices using a suction syringe. (scienceblogs.com)
- Using these mice, we found that the loss of DARPP-32 in striatonigral neurons decreased basal and cocaine-induced locomotion and abolished dyskinetic behaviors in response to the Parkinson's disease drug L-DOPA. (pnas.org)
- The most common known cause for both frontotemporal dementia and motor neuron disease is an unusual genetic mutation in the C9orf72 gene. (redorbit.com)
- Both RNA types accumulate into aggregates in the neurons of people with frontotemporal dementia. (redorbit.com)
- This correlation suggests that the build-up may be important in causing frontotemporal dementia and motor neuron disease, making the C9orf72 DNA expansion a potential target for therapy. (redorbit.com)
- Hardy J, Rogaeva E. Motor neuron disease and frontotemporal dementia: sometimes related, sometimes not. (medscape.com)
- Slow vertical saccades in the frontotemporal dementia with motor neuron disease. (medscape.com)
- Dynamic range adaptation in primary motor cortical populations , Robert G Rasmussen Andrew Schwartz Steven M Chase, eLife , doi: 10.7554/eLife.21409, published 18 April 2017. (medicalnewstoday.com)
- However, Ginsberg said, other motor neuron populations in the spinal cord could also contribute to the spinal cord's mitochondrial malady, and should be tested individually as well. (alzforum.org)
- Motor behaviors are the primary means by which animals interact with their environment, forming the final output of most central nervous system (CNS) activity. (nih.gov)
- This nervous system consists of the stomatogastric ganglion (STG), esophageal ganglion (OG) and commissural ganglia (CoGs), plus their connecting and motor nerves. (jneurosci.org)
- Motor neurons are responsible for passing information around the central nervous system (CNS) and from the CNS to the rest of the body. (sciencephoto.com)
- LOS ANGELES (March 23, 2018) -- A new study has revealed that the human brain's tiniest blood vessels can activate genes known to trigger spinal motor neurons, prompting the neurons to grow during early development. (eurekalert.org)
- Driven by developmental genes, some of these cells turn into spinal motor neurons. (eurekalert.org)
- The study showed the cells of the brain's smallest blood vessels, known as capillaries, are capable of activating these genes, which can spur spinal motor neurons to grow and mature. (eurekalert.org)
- The actions of a single family of transcription factors, encoded by the chromosomally clustered Hox genes, appear to have a central role in defining the specificity of motor neuron-muscle connectivity. (nih.gov)
- She sorted through 6,000 upper motor neuron genes that are vulnerable to ALS before she found one -- UCHL1 -- that is expressed through adulthood. (medindia.net)
- Led by Dr Angela Laird, this research team is focused on understanding how motor neurones die using the faulty genes that are identified in some patients with genetic or familial MND. (edu.au)
- Comparisons of model systems throughout the animal kingdom provide insights into general principles of motor control. (mit.edu)
- and sensory modification of motor output to control whole body orientation. (mit.edu)
- Every chapter has obviously beenwritten with the nonspecialist in mind, usually providing background andexplanations of technical terms that make the subject more accessible tostudents and to those in other fields who wish to understand current issuesin motor control. (mit.edu)
- It affects motor neurons that control the ability to talk, chew, and swallow. (webmd.com)
- In addition, she collected Purkinje neurons from unaffected brains to serve as another control sample. (alzforum.org)
- Motor neurons from both ALS and control samples had widely varying copy numbers, ranging from zero to 300,000. (alzforum.org)
- There are more α-MNs in the spinal cord than in the brainstem, as the number of α-MNs is directly proportional to the amount of fine motor control in that muscle. (wikipedia.org)
- SOD1(A4V/+) ALS patient-derived motor neurons have reduced delayed-rectifier potassium current amplitudes relative to control-derived motor neurons, a deficit that may underlie their hyperexcitability. (broadinstitute.org)
- The cervical and lumbosacral spinal cord segments are wider than the rest of the spinal cord because they contain the neurons that control the upper limbs and the lower limbs plus pelvic organs. (rutgers.edu)
- Stem cells can develop into many different types of cells, such as muscle cells, red blood cells, or neurons. (medicalnewstoday.com)
- New research discovers details involved in reprogramming stem cells into motor neurons. (medicalnewstoday.com)
- We have a very efficient system in which we can transform stem cells into motor neurons with something like a 90 to 95 percent success rate by adding the cocktail of transcription factors. (medicalnewstoday.com)
- To find out more about how scientists hope to use stem cells to better understand why motor neurons degenerate in people with ALS, read Dishing ALS . (als.net)
- The team has also demonstrated, in ALS motor neurons (pictured above), that mutated TDP-43 protein accumulates inside motor neurones and initiates degeneration in 95 per cent of ALS cases. (kcl.ac.uk)
- What are motor neurones? (hse.ie)
- Professor Julie Atkin leads investigations into the cellular responses to stress, and how these responses may enable early detection of injured motor neurones. (edu.au)
- There are two types of motor neurones. (mndnsw.asn.au)
- MND leads to the degeneration of these motor neurones. (mndnsw.asn.au)
- both upper and lower motor neurones. (mndnsw.asn.au)
- Most studies assume that individual M1 neurons encode time-invariant movement features within a direction, velocity, or force coordinate frame ( Scott, 2003 ). (jneurosci.org)
- Furthermore, they show that pathlets better capture the tuning properties of M1 neurons than previous models using time-invariant movement features. (jneurosci.org)
- Every voluntary movement is initiated and modulated by upper motor neurons -- answering a cell phone, typing an email, walking to the store. (medindia.net)
- In ALS, both the directing neurons and the neurons that create the movement disintegrate at the same time. (medindia.net)