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Cell Adhesion Molecules, Neuronal definition: Surface ligands that mediate cell-to-cell adhesion and function in the assembly and interconnection of the vertebrate nervous system. These molecules promote cell adhesion via a homophilic mechanism. These are not to be confused with NEURAL CELL ADHESION MOLECULES, now known to be expressed in a variety of tissues and cell types in addition to nervous tissue.
Synaptic Transmission definition: 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.
neural transmission definition: communication from a neuron to a target (neuron, muscle, or secretory cell) across a synapse.
transmission of nerve impulse definition: The sequential electrochemical polarization and depolarization that travels across the membrane of a nerve cell (neuron) in response to stimulation. [http://cancerweb.ncl.ac.uk]
synaptic transmission definition: The process of communication from a neuron to a target (neuron, muscle, or secretory cell) across a synapse. [GOC:jl, MeSH:D009435]
Neuronal Transmission definition: Neuronal Transmission is the intercellular propagation or transfer of a signal, from a neuron to a recipient cell, consisting of an electrical current by ion flux via gap junction or diffusion a chemical neurotransmitter across a synaptic or neuromuscular junction.
neuronal ceroid lipofuscinosis definition: inherited degenerative disease characterized by neuronal cytoplasmic inclusions which stain positively for ceroid and lipofuscin.
Neuronal Ceroid-Lipofuscinoses definition: A group of severe neurodegenerative diseases characterized by intracellular accumulation of autofluorescent wax-like lipid materials (CEROID; LIPOFUSCIN) in neurons. There are several subtypes based on mutations of the various genes, time of disease onset, and severity of the neurological defects such as progressive DEMENTIA; SEIZURES; and visual failure.
neuronal ceroid lipofuscinosis (CLN) definition: A group of inherited progressive neurometabolic diseases, previously considered as several separate syndromic entities, with considerable variability in clinical, pathological manifestations, and genetic findings. All diseases in this groups are characterized by abnormal storage of the autofluorescent proteolipopigments in neuronal and other structures with an average incidence estimated at 1:5/100,000. Six principal types are classified on the basis of age of onset, clinical manifestations, pathological changes, and genetic features. There appears to be a lack of uniformity in classification of CLN-5 and CLN-6. CLN-5 Boehme disease) is listed in OMIM in the Autosomal Dominant Catalog (OMIM: 162350) without a CLN number, and the late infantile variant as CLN- 5 (OMIM: 256731). CLN-7 has been added to include atypical forms of neuronal ceroid-lipofuscinosis that have not been included in previous classifications. Neuronal ceroid-lipofuscinosis type 1 (CLN-1) Synonyms: Hagberg-Santavuori disease Haltia-Santavuori disease Santavuori disease Santavuori-Haltia disease acute infantile neuronal ceroid-lipofuscinosis infantile Finnish type of neuronal ceroid-lipofuscinosis infantile neuronal ceroid-lipofuscinosis (INCL) polyunsaturated fatty acid lipidosis A variant encountered most frequently in Finland, hence the synonym infantile Finnish type of neuronal ceroid-lipofuscinosis, with onset at age 6 to 18 months and a subacute course. It is characterized by rapid deterioration with psychomotor retardation, loss of speech, seizures, ataxia, blindness, hypotonia, microcephaly, and occasional convulsions. Mapped to chromosome 1p32. The gene is known to be the palmityl-protein trioesterase. Transmitted as an autosomal recessive trait. (OMIM 256730) Neuronal ceroid-lipofuscinosis type 2 (CLN-2) Synonyms: Bernheimer-Seitelberger syndrome Bielschowsky amaurotic idiocy Bielschowsky disease Jansky-Bielschowsky disease Seitelberger disease late infantile amaurotic idiocy late infantile Batten disease late infantile neuronal lipofuscinosis (LINCL) subacute late infantile neuronal ceroid- lipofuscinosis The second most common variant with a subacute course after onset in infancy or early childhood characterized by refractive epilepsy, mental regression, ataxia, visual loss, and progressive deterioration. Mapped to chromosome 11p15. Pepstatin insensitive carboxypeptidase, 46dKa protein, Cys365->Tyr, Arg 208>umber stop codon, AG or intronic 3'spine junction to AC are the genetic features. Transmitted as an autosomal recessive trait. (OMIM 294500) neuronal ceroid-lipofuscinosis type 3 (CLN-3) Synonyms: Batten syndrome (BTS) Batten-Mayou syndrome Batten-Spielmeyer-Vogt disease Spielmeyer-Sjogren syndrome Spielmeyer-Vogt-Batten disease Spielmeyer-Vogt-Sjogren disease Stock-Spielmeyer-Vogt syndrome Vogt-Spielmeyer disease chronic juvenile neuronal ceroid-lipofuscinosis (JNCL) juvenile amaurotic family idiocy juvenile amaurotic idiocy juvenile Batten disease juvenile cerebrorenal degeneration juvenile neuronal lipofuscinosis (JNCL) juvenile onset neuronal ceroid-lipofuscinosis pigmentary retinal neuronal heredodegeneration The most commonly occurring variant with a chronic course after juvenile onset with an estimated incidence of 1:25,000. The first symptom is usually visual failure which takes place between the ages of 4 and 15 years. The early symptoms are followed by epilepsy and progressive physical and mental deterioration. Batten disease gene maps to chromosome 16p12.1. 56 chromosome haplotype defined by alleles at the D16S299 is shared by 73% of Batten disease chromosomes. Exon amplification of a cosmid containing D16S298 has yielded a candidate gene that is disrupted by kb genomic deletion in patients with 56 chromosomes. The disease gene encodes a 436 amino acid protein of unknown function. Transmitted as an autosomal recessive trait. (OMIM 294200) neuronal ceroid-lipofuscinosis type 4 (CLN-4) Synonyms: Kufs disease Kufs-Mayer disease adult amaurotic idiocy adult ceroid lipofuscinosis adult ganglioside lipidosis adult neuronal ceroid-lipofuscinosis adult recessive neuronal ceroid lipofuscinosis chronic adult-recessive neuronal ceroid-lipofuscinosis late familial amaurotic idiocy late ganglioside lipidosis A rare variant with onset of symptoms between the ages of 20 and 50 years with a chronic course and associated with cerebellar ataxia, bulbar symptoms, and extrapyramidal and pyramidal signs, but without retinal lesions and rapidly progressive dementia. Transmitted as an autosomal recessive trait but some cases are sporadic. (OMIM 204300) neuronal ceroid-lipofuscinosis type 5 (CLN-5) Synonyms: Boehme disease Parry neuronal ceroid-lipofuscinosis adult dominant neuronal ceroid-lipofuscinosis chronic adult dominant neuronal ceroid-lipofuscinosis dominant Kufs disease dominant neuronal ceroid-lipofuscinosis A cerebellar syndrome with onset early in fourth decade, characterized by epileptic fits, myoclonic epilepsy, progressive dementia, and hypertension. 11 cases were reported in four generations of a family named Parry. Transmitted as an autosomal dominant trait. (OMIM 162350) neuronal ceroid-lipofuscinosis type 6 (CLN-6) Synonyms: Zeman-Dyken-Lake-Santavuori-Savukoski disease subacute transitional early juvenile neuronal ceroid-lipofuscinosis A subacute variant with onset in late childhood or in early period with seizures, ataxia, retinal lesions, mental failure, and gradual neurological deterioration. neuronal ceroid-lipofuscinosis type 7 (NCL-7) A group of previously unclassified atypical forms of NCL, representing about 12 to 20% of those afflicted, characterized by accumulation of ceroid-lipofuscin in the secondary lysosomes or neurons and cells of other tissues, as skin, conjunctiva, and lymphocytes.
Neuronal Plasticity definition: The capacity of the NERVOUS SYSTEM to change its reactivity as the result of successive activations.
neural plasticity definition: ability of mature nerves and neurons to adapt their functional and morphological characteristics to environmental influences, such as during learning or compensation after loss.
Niemann Pick disease definition: caused by deficiency of sphingomyelinase, resulting in accumulation of sphingomyelin in the reticuloendothelial system, mental and physical retardation, enlarged liver and spleen, anemia, and blindness.
Niemann-Pick Diseases definition: A group of autosomal recessive disorders in which harmful quantities of lipids accumulate in the viscera and the central nervous system. They can be caused by deficiencies of enzyme activities (SPHINGOMYELIN PHOSPHODIESTERASE) or defects in intracellular transport, resulting in the accumulation of SPHINGOMYELINS and CHOLESTEROL. There are various subtypes based on their clinical and genetic differences.
neuronal transport definition: the movement of materials (subcellular organelles, cytoskeletal elements, soluble proteins) along the neuron from dendrites to cell body to axon and vice-versa; can be either fast or slow, anterograde or retrograde.
Infantile Neuronal Ceroid Lipofuscinosis definition: This type is caused by mutation in the gene (CLN1or PPT1) encoding palmitoyl-protein thioesterase (THIOLESTER HYDROLASES), an enzyme involved in catabolism of lipid-modified proteins.
Baculoviral IAP Repeat-Containing Protein 1 definition: Baculoviral IAP Repeat-Containing Protein 1, encoded by the BIRC1 gene, contains regions of homology to two baculovirus inhibitor of apoptosis proteins, It is able to suppress apoptosis induced by various signals and inhibits apoptosis in neuronal cells.
Neuronal Apoptosis-Inhibitory Protein definition: An inhibitor of apoptosis protein that was initially identified during analysis of CHROMOSOME DELETIONS associated with SPINAL MUSCULAR ATROPHY. Naip contains a nucleotide binding oligomerization domain and a carboxy-terminal LEUCINE rich repeat.
Neuronal and Mixed Neuronal-Glial Tumor definition: Neuronal and Mixed Neuronal-Glial Tumors have in common a variable amount of neuronal and, less consistently, glial differentiation. They occur at a low frequency and usually carry a favorable prognosis. They include: Gangliocytoma, Ganglioglioma, Dysembryoplastic Neuroepithelial Tumor, Central Neurocytoma, Cerebellar Liponeurocytoma and Paraganglioma. (Adapted from WHO.)
neuronal guidance definition: process by which developing or regenerating neurons extend their axons and/or dendrites to the "correct" target sites.
Juvenile Neuronal Ceroid Lipofuscinosis definition: This type is caused by mutation in the CLN3 gene encoding a lysosomal integral membrane protein (Battenin).
neurogenesis definition: Generation of cells within the nervous system. [GO_REF:0000021, GOC:cls, GOC:curators, GOC:dgh, GOC:dph, GOC:jic, GOC:mtg_15jun06]
neurogenesis definition: formation, maturation, growth, or differentiation of neurons, nerves, or neural tissue during development, regeneration, learning, or under experimental conditions.
Neurogenesis definition: Formation of NEURONS which involves the differentiation and division of STEM CELLS in which one or both of the daughter cells become neurons.
Neural Development definition: Neural Development involves proliferation, differentiation, and/or maturation of neural cells.
Nitric Oxide Synthase 1 definition: Encoded as 4 alternative isoforms by human NOS1 Gene (NOS Family), calcium/calmodulin stimulated homodimeric Nitric Oxide Synthase 1 binds FAD and FMN cofactors, contains a PDZ/DHR and a flavodoxin-like domain, requires BH4 which may stabilize the dimer, and synthesizes nitric oxide messenger from L-arginine. Neuronal NO displays neurotransmitter properties. N-truncated Isoform 3 possesses enzymatic activity. C-truncated isoform 4 is produced by frameshift exon insertion. NOS1 may interact with DLG4 and forms a ternary complex with CAPON and RASD1. NOS1 is localized beneath the sarcolemma in fast-twitch skeletal muscle fibers by associating with the dystrophin glycoprotein complex. The N-terminal PDZ domain of the neuronal NOS1 may participate in protein-protein interaction and target nNos to synaptic membranes. PIN inhibition may prevent NOS1 dimerization. (NCI)
synaptic target recognition definition: The process by which a neuronal cell in a multicellular organism interprets signals produced by potential target cells, with which it may form synapses. [GOC:mah, ISBN:0878932437]
neuron remodeling definition: The developmentally regulated remodeling of neuronal projections such as pruning to eliminate the extra dendrites and axons projections set up in early stages of nervous system development. [GOC:hb]
Neuronal SHC definition: Expressed in the brain by human SHC3 Gene, the 474-aa 52-kD and 594-aa 64-kD isoforms of Neuronal SHC are similar to SHC; nearly 70% homology in the PTB and SH2 domains. SHC3 appears involved in signaling from BDNF/TRKB to the RAS/MAPK pathway. BDNF induces tyrosine phosphorylation of SHC3 and binding to TRKB. Activated EGFR also interacts with and tyrosine-phosphorylates SHC3, inducing an association with GRB2. SHC3 and SCK mediate EGF signaling, but SHC3 has a higher affinity for TRKA and TRKB. (NCI)
neuron migration definition: The characteristic movement of immature neurons from germinal zones to specific positions where they will reside as they mature. [GOC:go_curators]
CDH2 Gene definition: This gene is involved in cell adhesion.
ENO2 Gene definition: This gene is involved in cell survival.
Neuronal Calcium-Sensor Proteins definition: A family of intracellular calcium-sensing proteins found predominately in NEURONS and PHOTORECEPTOR CELLS. They contain EF HAND MOTIFS and undergo conformational changes upon calcium-binding. Neuronal calcium-sensor proteins interact with other regulatory proteins to mediate physiological responses to a change in intracellular calcium concentration.
neuron apoptosis definition: The process of apoptosis in neurons, the basic cellular unit 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. [MeSH:A.08.663]
ensheathment of neuronal cell bodies definition: The process by which satellite glial cells isolate neuronal cell bodies. [GOC:dgh]
Neuronal Migration Disorders definition: Disorders resulting from defects in migration of neuronal cells during neurogenesis. Developing nerve cells either fail to migrate or they migrate to incorrect positions resulting in formation of heterotopias, lissencephaly, or other malformations and dysfunctions of the nervous system.