Myelin Sheath: The lipid-rich sheath surrounding AXONS in both the CENTRAL NERVOUS SYSTEMS and PERIPHERAL NERVOUS SYSTEM. The myelin sheath is an electrical insulator and allows faster and more energetically efficient conduction of impulses. The sheath is formed by the cell membranes of glial cells (SCHWANN CELLS in the peripheral and OLIGODENDROGLIA in the central nervous system). Deterioration of the sheath in DEMYELINATING DISEASES is a serious clinical problem.Myelin Proteins: MYELIN-specific proteins that play a structural or regulatory role in the genesis and maintenance of the lamellar MYELIN SHEATH structure.Myelin Basic Protein: An abundant cytosolic protein that plays a critical role in the structure of multilamellar myelin. Myelin basic protein binds to the cytosolic sides of myelin cell membranes and causes a tight adhesion between opposing cell membranes.Myelin P0 Protein: A protein that accounts for more than half of the peripheral nervous system myelin protein. The extracellular domain of this protein is believed to engage in adhesive interactions and thus hold the myelin membrane compact. It can behave as a homophilic adhesion molecule through interactions with its extracellular domains. (From J Cell Biol 1994;126(4):1089-97)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.Myelin Proteolipid Protein: A myelin protein that is the major component of the organic solvent extractable lipoprotein complexes of whole brain. It has been the subject of much study because of its unusual physical properties. It remains soluble in chloroform even after essentially all of its bound lipids have been removed. (From Siegel et al., Basic Neurochemistry, 4th ed, p122)Oligodendroglia: A class of large neuroglial (macroglial) cells in the central nervous system. Oligodendroglia may be called interfascicular, perivascular, or perineuronal (not the same as SATELLITE CELLS, PERINEURONAL of GANGLIA) according to their location. They form the insulating MYELIN SHEATH of axons in the central nervous system.Myelin-Associated Glycoprotein: A myelin protein found in the periaxonal membrane of both the central and peripheral nervous systems myelin sheaths. It binds to cells surface receptors found on AXONS and may regulate cellular interactions between MYELIN and AXONS.Demyelinating Diseases: Diseases characterized by loss or dysfunction of myelin in the central or peripheral nervous system.Schwann Cells: Neuroglial cells of the peripheral nervous system which form the insulating myelin sheaths of peripheral axons.Myelin-Oligodendrocyte Glycoprotein: A transmembrane protein present in the MYELIN SHEATH of the CENTRAL NERVOUS SYSTEM. It is one of the main autoantigens implicated in the pathogenesis of MULTIPLE SCLEROSIS.Axons: Nerve fibers that are capable of rapidly conducting impulses away from the neuron cell body.Glial Cell Line-Derived Neurotrophic Factor: The founding member of the glial cell line-derived neurotrophic factor family. It was originally characterized as a NERVE GROWTH FACTOR promoting the survival of MIDBRAIN dopaminergic NEURONS, and it has been studied as a potential treatment for PARKINSON DISEASE.Central Nervous System: The main information-processing organs of the nervous system, consisting of the brain, spinal cord, and meninges.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.Encephalomyelitis, Autoimmune, Experimental: An experimental animal model for central nervous system demyelinating disease. Inoculation with a white matter emulsion combined with FREUND'S ADJUVANT, myelin basic protein, or purified central myelin triggers a T cell-mediated immune response directed towards central myelin. The pathologic features are similar to MULTIPLE SCLEROSIS, including perivascular and periventricular foci of inflammation and demyelination. Subpial demyelination underlying meningeal infiltrations also occurs, which is also a feature of ENCEPHALOMYELITIS, ACUTE DISSEMINATED. Passive immunization with T-cells from an afflicted animal to a normal animal also induces this condition. (From Immunol Res 1998;17(1-2):217-27; Raine CS, Textbook of Neuropathology, 2nd ed, p604-5)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.Spinal Cord: A cylindrical column of tissue that lies within the vertebral canal. It is composed of WHITE MATTER and GRAY MATTER.Optic Nerve: The 2nd cranial nerve which conveys visual information from the RETINA to the brain. The nerve carries the axons of the RETINAL GANGLION CELLS which sort at the OPTIC CHIASM and continue via the OPTIC TRACTS to the brain. The largest projection is to the lateral geniculate nuclei; other targets include the SUPERIOR COLLICULI and the SUPRACHIASMATIC NUCLEI. Though known as the second cranial nerve, it is considered part of the CENTRAL NERVOUS SYSTEM.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.Multiple Sclerosis: An autoimmune disorder mainly affecting young adults and characterized by destruction of myelin in the central nervous system. Pathologic findings include multiple sharply demarcated areas of demyelination throughout the white matter of the central nervous system. Clinical manifestations include visual loss, extra-ocular movement disorders, paresthesias, loss of sensation, weakness, dysarthria, spasticity, ataxia, and bladder dysfunction. The usual pattern is one of recurrent attacks followed by partial recovery (see MULTIPLE SCLEROSIS, RELAPSING-REMITTING), but acute fulminating and chronic progressive forms (see MULTIPLE SCLEROSIS, CHRONIC PROGRESSIVE) also occur. (Adams et al., Principles of Neurology, 6th ed, p903)2',3'-Cyclic Nucleotide 3'-Phosphodiesterase: A phosphodiesterase that specifically cleaves the 3'-phosphate linkage of 2',3'-cyclic nucleotides. It is found at high level in the cytoplasm of cells that form the MYELIN SHEATH.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.Nerve Tissue ProteinsNerve Fibers, Myelinated: A class of nerve fibers as defined by their structure, specifically the nerve sheath arrangement. The AXONS of the myelinated nerve fibers are completely encased in a MYELIN SHEATH. They are fibers of relatively large and varied diameters. Their NEURAL CONDUCTION rates are faster than those of the unmyelinated nerve fibers (NERVE FIBERS, UNMYELINATED). Myelinated nerve fibers are present in somatic and autonomic nerves.Nerve Regeneration: Renewal or physiological repair of damaged nerve tissue.2',3'-Cyclic-Nucleotide Phosphodiesterases: Nucleoside-2',3'-cyclic phosphate nucleotidohydrolase. Enzymes that catalyze the hydrolysis of the 2'- or 3'- phosphate bonds of 2',3'-cyclic nucleotides. Also hydrolyzes nucleoside monophosphates. Includes EC 3.1.4.16 and EC 3.1.4.37. EC 3.1.4.-.Myelin P2 Protein: A positively charged protein found in peripheral nervous system MYELIN. Sensitive immunological techniques have demonstrated that P2 is expressed in small amounts of central nervous system myelin sheaths of some species. It is an antigen for experimental allergic neuritis (NEURITIS, EXPERIMENTAL ALLERGIC), the peripheral nervous system counterpart of experimental allergic encephalomyelitis. (From Siegel et al., Basic Neurochemistry, 5th ed, p133)Wallerian Degeneration: Degeneration of distal aspects of a nerve axon following injury to the cell body or proximal portion of the axon. The process is characterized by fragmentation of the axon and its MYELIN SHEATH.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.Sulfoglycosphingolipids: GLYCOSPHINGOLIPIDS with a sulfate group esterified to one of the sugar groups.Gliosis: The production of a dense fibrous network of neuroglia; includes astrocytosis, which is a proliferation of astrocytes in the area of a degenerative lesion.Mice, Jimpy: Myelin-deficient mutants which are from the inbred Tabby-Jimpy strain.Immunohistochemistry: Histochemical localization of immunoreactive substances using labeled antibodies as reagents.Ranvier's Nodes: Regularly spaced gaps in the myelin sheaths of peripheral axons. Ranvier's nodes allow saltatory conduction, that is, jumping of impulses from node to node, which is faster and more energetically favorable than continuous conduction.Glial Cell Line-Derived Neurotrophic Factor Receptors: A family of GLYCOSYLPHOSPHATIDYLINOSITOL-anchored cell surface receptors that are specific for GLIAL CELL LINE-DERIVED NEUROTROPHIC FACTORS. They form a multi-component receptor complex with PROTO-ONCOGENE PROTEIN C-RET and regulate a variety of intracellular SIGNAL TRANSDUCTION PATHWAYS in conjunction with c-ret protein.Microscopy, Electron: Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen.Cuprizone: Copper chelator that inhibits monoamine oxidase and causes liver and brain damage.Animals, Newborn: Refers to animals in the period of time just after birth.Cell Differentiation: Progressive restriction of the developmental potential and increasing specialization of function that leads to the formation of specialized cells, tissues, and organs.Microglia: The third type of glial cell, along with astrocytes and oligodendrocytes (which together form the macroglia). Microglia vary in appearance depending on developmental stage, functional state, and anatomical location; subtype terms include ramified, perivascular, ameboid, resting, and activated. Microglia clearly are capable of phagocytosis and play an important role in a wide spectrum of neuropathologies. They have also been suggested to act in several other roles including in secretion (e.g., of cytokines and neural growth factors), in immunological processing (e.g., antigen presentation), and in central nervous system development and remodeling.Brain Chemistry: Changes in the amounts of various chemicals (neurotransmitters, receptors, enzymes, and other metabolites) specific to the area of the central nervous system contained within the head. These are monitored over time, during sensory stimulation, or under different disease states.Mice, Inbred C57BLCharcot-Marie-Tooth Disease: A hereditary motor and sensory neuropathy transmitted most often as an autosomal dominant trait and characterized by progressive distal wasting and loss of reflexes in the muscles of the legs (and occasionally involving the arms). Onset is usually in the second to fourth decade of life. This condition has been divided into two subtypes, hereditary motor and sensory neuropathy (HMSN) types I and II. HMSN I is associated with abnormal nerve conduction velocities and nerve hypertrophy, features not seen in HMSN II. (Adams et al., Principles of Neurology, 6th ed, p1343)Mice, Transgenic: Laboratory mice that have been produced from a genetically manipulated EGG or EMBRYO, MAMMALIAN.Cerebrosides: Neutral glycosphingolipids that contain a monosaccharide, normally glucose or galactose, in 1-ortho-beta-glycosidic linkage with the primary alcohol of an N-acyl sphingoid (ceramide). In plants the monosaccharide is normally glucose and the sphingoid usually phytosphingosine. In animals, the monosaccharide is usually galactose, though this may vary with the tissue and the sphingoid is usually sphingosine or dihydrosphingosine. (From Oxford Dictionary of Biochemistry and Molecular Biology, 1st ed)Mice, Quaking: Mice homozygous for the mutant autosomal recessive gene, quaking (qk), associated with disorder in myelin formation and manifested by axial tremors.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.Ganglioside Galactosyltransferase: Catalyzes the final step in the galactocerebroside biosynthesis pathway.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.Nerve Growth Factors: Factors which enhance the growth potentialities of sensory and sympathetic nerve cells.Stem Cells: Relatively undifferentiated cells that retain the ability to divide and proliferate throughout postnatal life to provide progenitor cells that can differentiate into specialized cells.Glia Maturation Factor: A factor identified in the brain that influences the growth and differentiation of NEURONS and NEUROGLIA. Glia maturation factor beta is the 17-kDa polypeptide product of the GMFB gene and is the principal component of GLIA MATURATION FACTOR.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.N-Acylsphingosine Galactosyltransferase: An enzyme that catalyzes the conversion of UDP-galactose and N-acylsphingosine to D-galactosylceramide and UDP.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.Retina: The ten-layered nervous tissue membrane of the eye. It is continuous with the OPTIC NERVE and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the CHOROID and the inner surface with the VITREOUS BODY. The outer-most layer is pigmented, whereas the inner nine layers are transparent.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)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.Rats, Inbred LewCerebellum: 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.Early Growth Response Protein 2: An early growth response transcription factor that controls the formation of the MYELIN SHEATH around peripheral AXONS by SCHWANN CELLS. Mutations in EGR2 transcription factor have been associated with HEREDITARY MOTOR AND SENSORY NEUROPATHIES such as CHARCOT-MARIE-TOOTH DISEASE.Microscopy, Electron, Transmission: Electron microscopy in which the ELECTRONS or their reaction products that pass down through the specimen are imaged below the plane of the specimen.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.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.Amino Acid Sequence: The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.Peptide Fragments: Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.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.Demyelinating Autoimmune Diseases, CNS: Conditions characterized by loss or dysfunction of myelin (see MYELIN SHEATH) in the brain, spinal cord, or optic nerves secondary to autoimmune mediated processes. This may take the form of a humoral or cellular immune response directed toward myelin or OLIGODENDROGLIA associated autoantigens.S100 Proteins: A family of highly acidic calcium-binding proteins found in large concentration in the brain and believed to be glial in origin. They are also found in other organs in the body. They have in common the EF-hand motif (EF HAND MOTIFS) found on a number of calcium binding proteins. The name of this family derives from the property of being soluble in a 100% saturated ammonium sulfate solution.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.Corpus Callosum: Broad plate of dense myelinated fibers that reciprocally interconnect regions of the cortex in all lobes with corresponding regions of the opposite hemisphere. The corpus callosum is located deep in the longitudinal fissure.Neural Stem Cells: Self-renewing cells that generate the main phenotypes of the nervous system in both the embryo and adult. Neural stem cells are precursors to both NEURONS and NEUROGLIA.Cell Count: The number of CELLS of a specific kind, usually measured per unit volume or area of sample.Hereditary Sensory and Motor Neuropathy: A group of slowly progressive inherited disorders affecting motor and sensory peripheral nerves. Subtypes include HMSNs I-VII. HMSN I and II both refer to CHARCOT-MARIE-TOOTH DISEASE. HMSN III refers to hypertrophic neuropathy of infancy. HMSN IV refers to REFSUM DISEASE. HMSN V refers to a condition marked by a hereditary motor and sensory neuropathy associated with spastic paraplegia (see SPASTIC PARAPLEGIA, HEREDITARY). HMSN VI refers to HMSN associated with an inherited optic atrophy (OPTIC ATROPHIES, HEREDITARY), and HMSN VII refers to HMSN associated with retinitis pigmentosa. (From Adams et al., Principles of Neurology, 6th ed, p1343)Proteolipids: Protein-lipid combinations abundant in brain tissue, but also present in a wide variety of animal and plant tissues. In contrast to lipoproteins, they are insoluble in water, but soluble in a chloroform-methanol mixture. The protein moiety has a high content of hydrophobic amino acids. The associated lipids consist of a mixture of GLYCEROPHOSPHATES; CEREBROSIDES; and SULFOGLYCOSPHINGOLIPIDS; while lipoproteins contain PHOSPHOLIPIDS; CHOLESTEROL; and TRIGLYCERIDES.Nerve Crush: Treatment of muscles and nerves under pressure as a result of crush injuries.Glioma: Benign and malignant central nervous system neoplasms derived from glial cells (i.e., astrocytes, oligodendrocytes, and ependymocytes). Astrocytes may give rise to astrocytomas (ASTROCYTOMA) or glioblastoma multiforme (see GLIOBLASTOMA). Oligodendrocytes give rise to oligodendrogliomas (OLIGODENDROGLIOMA) and ependymocytes may undergo transformation to become EPENDYMOMA; CHOROID PLEXUS NEOPLASMS; or colloid cysts of the third ventricle. (From Escourolle et al., Manual of Basic Neuropathology, 2nd ed, p21)SOXE Transcription Factors: A subclass of closely-related SOX transcription factors. Members of this subfamily have been implicated in regulating the differentiation of OLIGODENDROCYTES during neural crest formation and in CHONDROGENESIS.Peripheral Nervous System Diseases: Diseases of the peripheral nerves external to the brain and spinal cord, which includes diseases of the nerve roots, ganglia, plexi, autonomic nerves, sensory nerves, and motor nerves.S100 Calcium Binding Protein beta Subunit: A calcium-binding protein that is 92 AA long, contains 2 EF-hand domains, and is concentrated mainly in GLIAL CELLS. Elevation of S100B levels in brain tissue correlates with a role in neurological disorders.Nestin: A type VI intermediate filament protein expressed mostly in nerve cells where it is associated with the survival, renewal and mitogen-stimulated proliferation of neural progenitor cells.Time Factors: Elements of limited time intervals, contributing to particular results or situations.Spinal Cord Injuries: Penetrating and non-penetrating injuries to the spinal cord resulting from traumatic external forces (e.g., WOUNDS, GUNSHOT; WHIPLASH INJURIES; etc.).Cell Adhesion Molecules, Neuronal: 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.HLA-DR2 Antigen: A broad specificity HLA-DR antigen that is associated with HLA-DRB1 CHAINS encoded by DRB1*01:15 and DRB1*01:16 alleles.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.Fluorescent Antibody Technique: Test for tissue antigen using either a direct method, by conjugation of antibody with fluorescent dye (FLUORESCENT ANTIBODY TECHNIQUE, DIRECT) or an indirect method, by formation of antigen-antibody complex which is then labeled with fluorescein-conjugated anti-immunoglobulin antibody (FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT). The tissue is then examined by fluorescence microscopy.Neural Conduction: The propagation of the NERVE IMPULSE along the nerve away from the site of an excitation stimulus.Neuritis, Autoimmune, Experimental: An experimental animal model for the demyelinating disease of GUILLAINE-BARRE SYNDROME. In the most frequently used protocol, animals are injected with a peripheral nerve tissue protein homogenate. After approximately 2 weeks the animals develop a neuropathy secondary to a T cell-mediated autoimmune response directed towards the MYELIN P2 PROTEIN in peripheral nerves. Pathologic findings include a perivascular accumulation of macrophages and T lymphocytes in the peripheral nervous system, similar to that seen in the Guillaine-Barre syndrome. (From Adams et al., Principles of Neurology, 6th ed, p1314; J Neuroimmunol 1998 Apr 1;84(1):40-52)Aquaporin 4: Aquaporin 4 is the major water-selective channel in the CENTRAL NERVOUS SYSTEM of mammals.Sural Nerve: A branch of the tibial nerve which supplies sensory innervation to parts of the lower leg and foot.Vimentin: An intermediate filament protein found in most differentiating cells, in cells grown in tissue culture, and in certain fully differentiated cells. Its insolubility suggests that it serves a structural function in the cytoplasm. MW 52,000.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.Intermediate Filament Proteins: Filaments 7-11 nm in diameter found in the cytoplasm of all cells. Many specific proteins belong to this group, e.g., desmin, vimentin, prekeratin, decamin, skeletin, neurofilin, neurofilament protein, and glial fibrillary acid protein.Blotting, Western: Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.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.Amino Acid Transport System X-AG: A family of POTASSIUM and SODIUM-dependent acidic amino acid transporters that demonstrate a high affinity for GLUTAMIC ACID and ASPARTIC ACID. Several variants of this system are found in neuronal tissue.Coculture Techniques: A technique of culturing mixed cell types in vitro to allow their synergistic or antagonistic interactions, such as on CELL DIFFERENTIATION or APOPTOSIS. Coculture can be of different types of cells, tissues, or organs from normal or disease states.Diffuse Cerebral Sclerosis of Schilder: A rare central nervous system demyelinating condition affecting children and young adults. Pathologic findings include a large, sharply defined, asymmetric focus of myelin destruction that may involve an entire lobe or cerebral hemisphere. The clinical course tends to be progressive and includes dementia, cortical blindness, cortical deafness, spastic hemiplegia, and pseudobulbar palsy. Concentric sclerosis of Balo is differentiated from diffuse cerebral sclerosis of Schilder by the pathologic finding of alternating bands of destruction and preservation of myelin in concentric rings. Alpers' Syndrome refers to a heterogeneous group of diseases that feature progressive cerebral deterioration and liver disease. (From Adams et al., Principles of Neurology, 6th ed, p914; Dev Neurosci 1991;13(4-5):267-73)Cell Communication: Any of several ways in which living cells of an organism communicate with one another, whether by direct contact between cells or by means of chemical signals carried by neurotransmitter substances, hormones, and cyclic AMP.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)Excitatory Amino Acid Transporter 1: A glial type glutamate plasma membrane transporter protein found predominately in ASTROCYTES. It is also expressed in HEART and SKELETAL MUSCLE and in the PLACENTA.Cell Lineage: The developmental history of specific differentiated cell types as traced back to the original STEM CELLS in the embryo.Aging: The gradual irreversible changes in structure and function of an organism that occur as a result of the passage of time.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 Movement: The movement of cells from one location to another. Distinguish from CYTOKINESIS which is the process of dividing the CYTOPLASM of a cell.Astrocytoma: Neoplasms of the brain and spinal cord derived from glial cells which vary from histologically benign forms to highly anaplastic and malignant tumors. Fibrillary astrocytomas are the most common type and may be classified in order of increasing malignancy (grades I through IV). In the first two decades of life, astrocytomas tend to originate in the cerebellar hemispheres; in adults, they most frequently arise in the cerebrum and frequently undergo malignant transformation. (From Devita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2013-7; Holland et al., Cancer Medicine, 3d ed, p1082)Mice, Inbred Strains: Genetically identical individuals developed from brother and sister matings which have been carried out for twenty or more generations, or by parent x offspring matings carried out with certain restrictions. All animals within an inbred strain trace back to a common ancestor in the twentieth generation.Hereditary Central Nervous System Demyelinating Diseases: Inherited conditions characterized by a loss of MYELIN in the central nervous system.Galactosylceramides: Cerebrosides which contain as their polar head group a galactose moiety bound in glycosidic linkage to the hydroxyl group of ceramide. Their accumulation in tissue, due to a defect in beta-galactosidase, is the cause of galactosylceramide lipidosis or globoid cell leukodystrophy.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.Brain Neoplasms: Neoplasms of the intracranial components of the central nervous system, including the cerebral hemispheres, basal ganglia, hypothalamus, thalamus, brain stem, and cerebellum. Brain neoplasms are subdivided into primary (originating from brain tissue) and secondary (i.e., metastatic) forms. Primary neoplasms are subdivided into benign and malignant forms. In general, brain tumors may also be classified by age of onset, histologic type, or presenting location in the brain.Satellite Cells, Perineuronal: The non-neuronal cells that surround the neuronal cell bodies of the GANGLIA. They are distinguished from the perineuronal satellite oligodendrocytes (OLIGODENDROGLIA) found in the central nervous system.Proto-Oncogene Proteins c-ret: Receptor protein-tyrosine kinases involved in the signaling of GLIAL CELL-LINE DERIVED NEUROTROPHIC FACTOR ligands. They contain an extracellular cadherin domain and form a receptor complexes with GDNF RECEPTORS. Mutations in ret protein are responsible for HIRSCHSPRUNG DISEASE and MULTIPLE ENDOCRINE NEOPLASIA TYPE 2.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.Neuregulin-1: A peptide factor originally identified by its ability to stimulate the phosphorylation the erbB-2 receptor (RECEPTOR, ERBB-2). It is a ligand for the erbB-3 receptor (RECEPTOR, ERBB-3) and the erbB-4 receptor. Variant forms of NEUREGULIN-1 occur through alternative splicing of its mRNA.Cell Proliferation: All of the processes involved in increasing CELL NUMBER including CELL DIVISION.Neurogenesis: Formation of NEURONS which involves the differentiation and division of STEM CELLS in which one or both of the daughter cells become neurons.Phosphopyruvate Hydratase: A hydro-lyase that catalyzes the dehydration of 2-phosphoglycerate to form PHOSPHOENOLPYRUVATE. Several different isoforms of this enzyme exist, each with its own tissue specificity.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.Ependymoglial Cells: The macroglial cells of EPENDYMA. They are characterized by bipolar cell body shape and processes that contact BASAL LAMINA around blood vessels and/or the PIA MATER and the CEREBRAL VENTRICLES.T-Lymphocytes: Lymphocytes responsible for cell-mediated immunity. Two types have been identified - cytotoxic (T-LYMPHOCYTES, CYTOTOXIC) and helper T-lymphocytes (T-LYMPHOCYTES, HELPER-INDUCER). They are formed when lymphocytes circulate through the THYMUS GLAND and differentiate to thymocytes. When exposed to an antigen, they divide rapidly and produce large numbers of new T cells sensitized to that antigen.Glial Cell Line-Derived Neurotrophic Factors: A family of closely related nerve growth factors that promote NEURON survival. They bind to GDNF RECEPTORS and stimulate SIGNAL TRANSDUCTION through PROTO-ONCOGENE PROTEIN C-RET.Gene Expression: The phenotypic manifestation of a gene or genes by the processes of GENETIC TRANSCRIPTION and GENETIC TRANSLATION.Base Sequence: The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.Autoantigens: Endogenous tissue constituents that have the ability to interact with AUTOANTIBODIES and cause an immune response.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.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.Bromodeoxyuridine: A nucleoside that substitutes for thymidine in DNA and thus acts as an antimetabolite. It causes breaks in chromosomes and has been proposed as an antiviral and antineoplastic agent. It has been given orphan drug status for use in the treatment of primary brain tumors.Cattle: Domesticated bovine animals of the genus Bos, usually kept on a farm or ranch and used for the production of meat or dairy products or for heavy labor.Retinal Ganglion Cells: Neurons of the innermost layer of the retina, the internal plexiform layer. They are of variable sizes and shapes, and their axons project via the OPTIC NERVE to the brain. A small subset of these cells act as photoreceptors with projections to the SUPRACHIASMATIC NUCLEUS, the center for regulating CIRCADIAN RHYTHM.Galactolipids: A group of GLYCOLIPIDS in which the sugar group is GALACTOSE. They are distinguished from GLYCOSPHINGOLIPIDS in lacking nitrogen. They constitute the majority of MEMBRANE LIPIDS in PLANTS.Drosophila Proteins: Proteins that originate from insect species belonging to the genus DROSOPHILA. The proteins from the most intensely studied species of Drosophila, DROSOPHILA MELANOGASTER, are the subject of much interest in the area of MORPHOGENESIS and development.Excitatory Amino Acid Transporter 2: A glutamate plasma membrane transporter protein found in ASTROCYTES and in the LIVER.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.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.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.Reverse Transcriptase Polymerase Chain Reaction: A variation of the PCR technique in which cDNA is made from RNA via reverse transcription. The resultant cDNA is then amplified using standard PCR protocols.Glial Fibrillary Acidic Protein: An intermediate filament protein found only in glial cells or cells of glial origin. MW 51,000.Transcription Factors: Endogenous substances, usually proteins, which are effective in the initiation, stimulation, or termination of the genetic transcription process.Rats, Inbred Strains: Genetically identical individuals developed from brother and sister matings which have been carried out for twenty or more generations or by parent x offspring matings carried out with certain restrictions. This also includes animals with a long history of closed colony breeding.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)Alexander Disease: Rare leukoencephalopathy with infantile-onset accumulation of Rosenthal fibers in the subpial, periventricular, and subependymal zones of the brain. Rosenthal fibers are GLIAL FIBRILLARY ACIDIC PROTEIN aggregates found in ASTROCYTES. Juvenile- and adult-onset types show progressive atrophy of the lower brainstem instead. De novo mutations in the GFAP gene are associated with the disease with propensity for paternal inheritance.Phenotype: The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.Glycoproteins: Conjugated protein-carbohydrate compounds including mucins, mucoid, and amyloid glycoproteins.Cell Division: The fission of a CELL. It includes CYTOKINESIS, when the CYTOPLASM of a cell is divided, and CELL NUCLEUS DIVISION.Ependyma: A thin membrane that lines the CEREBRAL VENTRICLES and the central canal of the SPINAL CORD.Neuritis: A general term indicating inflammation of a peripheral or cranial nerve. Clinical manifestation may include PAIN; PARESTHESIAS; PARESIS; or HYPESTHESIA.Fluorescent Antibody Technique, Indirect: A form of fluorescent antibody technique commonly used to detect serum antibodies and immune complexes in tissues and microorganisms in specimens from patients with infectious diseases. The technique involves formation of an antigen-antibody complex which is labeled with fluorescein-conjugated anti-immunoglobulin antibody. (From Bennington, Saunders Dictionary & Encyclopedia of Laboratory Medicine and Technology, 1984)GPI-Linked Proteins: A subclass of lipid-linked proteins that contain a GLYCOSYLPHOSPHATIDYLINOSITOL LINKAGE which holds them to the CELL MEMBRANE.Mice, Mutant Strains: Mice bearing mutant genes which are phenotypically expressed in the animals.Electrophoresis, Polyacrylamide Gel: Electrophoresis in which a polyacrylamide gel is used as the diffusion medium.Nerve Fibers: Slender processes of NEURONS, including the AXONS and their glial envelopes (MYELIN SHEATH). Nerve fibers conduct nerve impulses to and from the CENTRAL NERVOUS SYSTEM.Protein Isoforms: Different forms of a protein that may be produced from different GENES, or from the same gene by ALTERNATIVE SPLICING.Telencephalon: The anterior subdivision of the embryonic PROSENCEPHALON or the corresponding part of the adult prosencephalon that includes the cerebrum and associated structures.Enteric Nervous System: Two ganglionated neural plexuses in the gut wall which form one of the three major divisions of the autonomic nervous system. The enteric nervous system innervates the gastrointestinal tract, the pancreas, and the gallbladder. It contains sensory neurons, interneurons, and motor neurons. Thus the circuitry can autonomously sense the tension and the chemical environment in the gut and regulate blood vessel tone, motility, secretions, and fluid transport. The system is itself governed by the central nervous system and receives both parasympathetic and sympathetic innervation. (From Kandel, Schwartz, and Jessel, Principles of Neural Science, 3d ed, p766)Cytokines: Non-antibody proteins secreted by inflammatory leukocytes and some non-leukocytic cells, that act as intercellular mediators. They differ from classical hormones in that they are produced by a number of tissue or cell types rather than by specialized glands. They generally act locally in a paracrine or autocrine rather than endocrine manner.Optic Nerve Injuries: Injuries to the optic nerve induced by a trauma to the face or head. These may occur with closed or penetrating injuries. Relatively minor compression of the superior aspect of orbit may also result in trauma to the optic nerve. Clinical manifestations may include visual loss, PAPILLEDEMA, and an afferent pupillary defect.Oligodendroglioma: A relatively slow-growing glioma that is derived from oligodendrocytes and tends to occur in the cerebral hemispheres, thalamus, or lateral ventricle. They may present at any age, but are most frequent in the third to fifth decades, with an earlier incidence peak in the first decade. Histologically, these tumors are encapsulated, relatively avascular, and tend to form cysts and microcalcifications. Neoplastic cells tend to have small round nuclei surrounded by unstained nuclei. The tumors may vary from well-differentiated to highly anaplastic forms. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, p2052; Adams et al., Principles of Neurology, 6th ed, p655)Cell Death: The termination of the cell's ability to carry out vital functions such as metabolism, growth, reproduction, responsiveness, and adaptability.Encephalomyelitis: A general term indicating inflammation of the BRAIN and SPINAL CORD, often used to indicate an infectious process, but also applicable to a variety of autoimmune and toxic-metabolic conditions. There is significant overlap regarding the usage of this term and ENCEPHALITIS in the literature.Contactins: A family of immunoglobulin-related cell adhesion molecules that are involved in NERVOUS SYSTEM patterning.Animals, Genetically Modified: ANIMALS whose GENOME has been altered by GENETIC ENGINEERING, or their offspring.Sciatic Neuropathy: Disease or damage involving the SCIATIC NERVE, which divides into the PERONEAL NERVE and TIBIAL NERVE (see also PERONEAL NEUROPATHIES and TIBIAL NEUROPATHY). Clinical manifestations may include SCIATICA or pain localized to the hip, PARESIS or PARALYSIS of posterior thigh muscles and muscles innervated by the peroneal and tibial nerves, and sensory loss involving the lateral and posterior thigh, posterior and lateral leg, and sole of the foot. The sciatic nerve may be affected by trauma; ISCHEMIA; COLLAGEN DISEASES; and other conditions. (From Adams et al., Principles of Neurology, 6th ed, p1363)Antigens: Substances that are recognized by the immune system and induce an immune reaction.Cell Membrane: The lipid- and protein-containing, selectively permeable membrane that surrounds the cytoplasm in prokaryotic and eukaryotic cells.Blood-Brain Barrier: Specialized non-fenestrated tightly-joined ENDOTHELIAL CELLS with TIGHT JUNCTIONS that form a transport barrier for certain substances between the cerebral capillaries and the BRAIN tissue.Peripheral Nerve Injuries: Injuries to the PERIPHERAL NERVES.Peptides: Members of the class of compounds composed of AMINO ACIDS joined together by peptide bonds between adjacent amino acids into linear, branched or cyclical structures. OLIGOPEPTIDES are composed of approximately 2-12 amino acids. Polypeptides are composed of approximately 13 or more amino acids. PROTEINS are linear polypeptides that are normally synthesized on RIBOSOMES.Cicatrix: The fibrous tissue that replaces normal tissue during the process of WOUND HEALING.Neurturin: A glial cell line-derived neurotrophic factor ligand that is specific for the GFRA2 RECEPTOR. Neurturin is essential for the development of specific postganglionic parasympathetic NEURONS.Immunoenzyme Techniques: Immunologic techniques based on the use of: (1) enzyme-antibody conjugates; (2) enzyme-antigen conjugates; (3) antienzyme antibody followed by its homologous enzyme; or (4) enzyme-antienzyme complexes. These are used histologically for visualizing or labeling tissue specimens.Phosphorylation: The introduction of a phosphoryl group into a compound through the formation of an ester bond between the compound and a phosphorus moiety.Magnetic Resonance Imaging: Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques.Ganglioglioma: Rare indolent tumors comprised of neoplastic glial and neuronal cells which occur primarily in children and young adults. Benign lesions tend to be associated with long survival unless the tumor degenerates into a histologically malignant form. They tend to occur in the optic nerve and white matter of the brain and spinal cord.Biological Markers: Measurable and quantifiable biological parameters (e.g., specific enzyme concentration, specific hormone concentration, specific gene phenotype distribution in a population, presence of biological substances) which serve as indices for health- and physiology-related assessments, such as disease risk, psychiatric disorders, environmental exposure and its effects, disease diagnosis, metabolic processes, substance abuse, pregnancy, cell line development, epidemiologic studies, etc.Autoimmunity: Process whereby the immune system reacts against the body's own tissues. Autoimmunity may produce or be caused by AUTOIMMUNE DISEASES.Microscopy, Immunoelectron: Microscopy in which the samples are first stained immunocytochemically and then examined using an electron microscope. Immunoelectron microscopy is used extensively in diagnostic virology as part of very sensitive immunoassays.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.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.Cerebral Ventricles: Four CSF-filled (see CEREBROSPINAL FLUID) cavities within the cerebral hemispheres (LATERAL VENTRICLES), in the midline (THIRD VENTRICLE) and within the PONS and MEDULLA OBLONGATA (FOURTH VENTRICLE).Histocytochemistry: Study of intracellular distribution of chemicals, reaction sites, enzymes, etc., by means of staining reactions, radioactive isotope uptake, selective metal distribution in electron microscopy, or other methods.Drosophila: A genus of small, two-winged flies containing approximately 900 described species. These organisms are the most extensively studied of all genera from the standpoint of genetics and cytology.Membrane Proteins: Proteins which are found in membranes including cellular and intracellular membranes. They consist of two types, peripheral and integral proteins. They include most membrane-associated enzymes, antigenic proteins, transport proteins, and drug, hormone, and lectin receptors.Freeze Fracturing: Preparation for electron microscopy of minute replicas of exposed surfaces of the cell which have been ruptured in the frozen state. The specimen is frozen, then cleaved under high vacuum at the same temperature. The exposed surface is shadowed with carbon and platinum and coated with carbon to obtain a carbon replica.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.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.Protein Binding: The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.Antibodies: Immunoglobulin molecules having a specific amino acid sequence by virtue of which they interact only with the ANTIGEN (or a very similar shape) that induced their synthesis in cells of the lymphoid series (especially PLASMA CELLS).Organ Culture Techniques: A technique for maintenance or growth of animal organs in vitro. It refers to three-dimensional cultures of undisaggregated tissue retaining some or all of the histological features of the tissue in vivo. (Freshney, Culture of Animal Cells, 3d ed, p1)Kinetics: The rate dynamics in chemical or physical systems.Neuropeptides: Peptides released by NEURONS as intercellular messengers. Many neuropeptides are also hormones released by non-neuronal cells.Transfection: The uptake of naked or purified DNA by CELLS, usually meaning the process as it occurs in eukaryotic cells. It is analogous to bacterial transformation (TRANSFORMATION, BACTERIAL) and both are routinely employed in GENE TRANSFER TECHNIQUES.Lymphocyte Activation: Morphologic alteration of small B LYMPHOCYTES or T LYMPHOCYTES in culture into large blast-like cells able to synthesize DNA and RNA and to divide mitotically. It is induced by INTERLEUKINS; MITOGENS such as PHYTOHEMAGGLUTININS, and by specific ANTIGENS. It may also occur in vivo as in GRAFT REJECTION.Brain Tissue Transplantation: Transference of brain tissue, either from a fetus or from a born individual, between individuals of the same species or between individuals of different species.Recovery of Function: A partial or complete return to the normal or proper physiologic activity of an organ or part following disease or trauma.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.Epitopes: Sites on an antigen that interact with specific antibodies.Brain Injuries: Acute and chronic (see also BRAIN INJURIES, CHRONIC) injuries to the brain, including the cerebral hemispheres, CEREBELLUM, and BRAIN STEM. Clinical manifestations depend on the nature of injury. Diffuse trauma to the brain is frequently associated with DIFFUSE AXONAL INJURY or COMA, POST-TRAUMATIC. Localized injuries may be associated with NEUROBEHAVIORAL MANIFESTATIONS; HEMIPARESIS, or other focal neurologic deficits.Motor Neurons: Neurons which activate MUSCLE CELLS.
The glial cells express a loss of myelin. This loss of myelin is different from that seen in other diseases where ... In affected cells, the protein is reduced to about 50%, which is acceptable for functionality in most cells, but not in glial ... A unique characteristic of VWM is that only oligodendrocytes and astrocytes are negatively affected while other glial cells and ... Melatonin has been shown to provide cytoprotective traits to glial cells exposed to stressors such as excitotoxicity and ...
Schwann cells are glial cells that myelinate the axons of nerve cells. Myelin is a lipid covering that speeds the conduction of ...
Consequently, a reduction of glial-derived oligodendroctyes, among others, compromise maintenance of myelin production for axon ... Subsequent differentiation of glial cell populations results in function-specialized glial lineages. Glial cell-derived ... 1997) Glial ontogeny and glial neoplasia: The search for closure. Journal of Neuro-Oncology 34: 5-22. PMID 9210049. Barres BA, ... Functions of glial-derived cell lineages are reviewed by Baumann and Hauw. Gliogenesis itself, and differentiation of glial- ...
Lipid metabolism, necessary for myelin production. Amino acid modification. Probably linked to the production of taurine, of ... Thiamine deficiency affects both neurons and astrocytes, glial cells of the brain. Thiamine deficiency alters the glutamate ...
MAG -Myelin-associated glycoprotein acts via the receptors NgR2, GT1b, NgR1, p75, TROY and LINGO1. OMgp -Oligodendrocyte Myelin ... Oligodendrocyte precursor cells are another type of glial cell found in the central nervous system that play a role in glial ... The resulting analysis showed that the GAG profiles of normal cortex and glial scar tissue were significantly different. Glial ... Glial scar formation is induced following damage to the nervous system. In the central nervous system, this glial scar ...
Myelin is a layer of a fatty insulating substance, which is formed by two types of glial cells Schwann cells and ... In the central nervous system the myelin sheath is provided by another type of glial cell, the oligodendrocyte. Schwann cells ... In the central nervous system oligodendrocytes form the insulating myelin. Along myelinated nerve fibers, gaps in the myelin ... The myelin gives the white appearance to the tissue in contrast to the grey matter of the cerebral cortex which contains the ...
Glial cells maintain homeostasis, produce myelin, and provide support and protection for the brain's neurons. Some glial cells ... At the tissue level, the nervous system is composed of neurons, glial cells, and extracellular matrix. Both neurons and glial ... glial processes or blood vessels, or specific intracytoplasmic or intranuclear proteins and other immunogenetic molecules, e.g ...
Oligodendrocytes are glial cells that produce a fatty substance called myelin, which wraps around axons in layers. Axon fibers ... Glial cells are "nerve-helper" cells that provide structural support, nourishment and protection for neurons. Astrocytes, a ... A spinal cord injury can compromise or destroy myelin. Messages are passed from neuron to neuron through synapses, small gaps ... 2. The death of oligodendrocytes causes axons to lose their myelin, which greatly impairs the conduction of messages or renders ...
Myelin allows the fast propagation of electrical impulses, but glial cells are also required for axonal maintenance. This line ... A major focus of their research is on neuron-glia interactions that result in the assembly of myelin in the nervous system. ... These highly specialized glial cells wrap and electrically insulate axons in the peripheral and central nervous system, ... research helps us to understand the molecular mechanisms of human neurological diseases in which genetic mutations cause myelin ...
... and neurofilament to characterize axonal changes and myelin basic protein for myelin pathology. Immunohistochemical stains for ... In severely degenerated areas there are many large, reactive astrocytes filled with glial fibrils. In autopsy cases, it has ... Special stains for myelin and axonal pathology show the abnormal changes that are characteristic of HDLS are identified in ... The loss of white matter is caused by myelin loss. These changes are associated with diffuse gliosis, moderate loss of axons ...
It also causes an overload of long chain fatty acids in the brain which destroy the myelin sheath. The cause of Alexander ... It is caused by mutations in the gene for glial fibrillary acidic protein (GFAP) that maps to chromosome 17q21. It is inherited ... The Myelin Project The Stennis Foundation "MUTATION KEY TO ALEXANDER DISEASE" - United Press International GeneReviews/NCBI/NIH ... Alexander disease belongs to leukodystrophies, a group of diseases that affect the growth or development of the myelin sheath. ...
1994). "Glial growth factor restricts mammalian neural crest stem cells to glial fate". Cell. 77: 349-360. doi:10.1016/0092- ... Myelin protein zero (P0) is a cell adhesion molecule belonging to the immunoglobulin superfamily and is the major component of ... A well-developed Schwann cell is shaped like a rolled-up sheet of paper, with layers of myelin in between each coil. The inner ... P0 has been shown to be essential for the formation of compact myelin, as P0 null mutant (P0-) mice showed severely aberrant ...
In this disease the body attacks the glial cells that form myelin coats around the axons on neurons. This causes the nervous ...
Schwann cells are glial cells in the peripheral nervous system that support neurons by forming myelin that encases nerves. ... myelin is fragmented and myelin debris and lipid droplets are found in the cytoplasm of Schwann cells, indicating phagocytic ... They not only have a role in phagocytosis of myelin, but they also have a role in recruitment of macrophages to continue the ... In this case, the axon remains intact, but there is myelin damage causing an interruption in conduction of the impulse down the ...
Subsequent waves of neurons split the preplate by migrating along radial glial fibres to form the cortical plate. Each wave of ... myelin or synapses. Neuroregeneration differs between the peripheral nervous system (PNS) and the central nervous system (CNS) ... It is estimated that glial guided migration represents 80-90% of migrating neurons.[citation needed] Many neurons migrating ... Neuroscience portal Time lapse sequences of radial migration (also known as glial guidance) and somal translocation. Axon ...
Demyelination is the act of demyelinating, or the loss of the myelin sheath insulating the nerves. When myelin degrades, ... For example, glial cells, which are not considered neurons, play an essential role in information processing. Also, electrical ... The myelin sheath in peripheral nerves normally runs along the axon in sections about 1 mm long, punctuated by unsheathed nodes ... Astrocytes are star-shaped glial cells that have also been observed to turn into neurons by virtue of the stem cell ...
... the glial cell responsible for maintaining myelin. Changes in white matter known as amyloid plaques are associated with ... The white matter is white because of the fatty substance (myelin) that surrounds the nerve fibers (axons). This myelin is found ... Myelin acts as an insulator, which allows electrical signals to jump, rather than coursing through the axon, increasing the ... However, the tissue of the freshly cut brain appears pinkish white to the naked eye because myelin is composed largely of lipid ...
... , also called glial cells or simply glia, are non-neuronal cells that maintain homeostasis, form myelin, and provide ... Each "arm" reaches out to a nerve fiber and spirals around it, creating a myelin sheath. This myelin sheath insulates the nerve ... These PNS glia include Schwann cells in nerves and satellite glial cells in ganglia. Current research involving glial cells in ... Polydendrocytes Jessen KR, Mirsky R (August 1980). "Glial cells in the enteric nervous system contain glial fibrillary acidic ...
The root exit zone is where the central glial axonal insulation of the nerve ends and the peripheral nerve axonal myelination ... Biopsies of the root exit zone demonstrated degeneration of axons, denuded axis cylinder and interrupted myelin. The results of ...
The notion that there exists a population of glial progenitor cells in the developing and mature CNS began to be entertained in ... Peru, R. L.; Mandrycky, N.; Nait-Oumesmar, B.; Lu, Q. R. (2008). "Paving the Axonal Highway: From Stem Cells to Myelin Repair ... Raff, M. C.; Miller, R. H.; Noble, M. (1983). "A glial progenitor cell that develops in vitro into an astrocyte or an ... Kang, S. H.; Fukaya, M.; Yang, J. K.; Rothstein, J. D.; Bergles, D. E. (2010). "NG2+ CNS Glial Progenitors Remain Committed to ...
Myelin also increases impulse speed, as saltatory propagation of action potentials occurs at the nodes of Ranvier in between ... Oligodendroglia, types of glial cells, arise during development from oligodendrocyte precursor cells, which can be identified ... The optimal ratio of axon diameter divided by the total fiber diameter (which includes the myelin) is 0.6. In contrast, ... A single oligodendrocyte can extend its processes to 50 axons, wrapping approximately 1 μm of myelin sheath around each axon; ...
When glial cells and TLRs are negatively affected they are not able to react to developing inflammation in the brain as well. ... However, in regions of grey matter, large dense aggregates of myelin are present. Ulegyria affected neurons also display ... This study suggested that intrauterine infections in the mother could affect glial cells and toll-like receptors (TLRs) which ... For instance, ulegyria affected neurons experience gliosis in which glial cells, specifically astrocytes, build up near and ...
The axon is insulated by a myelin sheath. Myelin is composed of either Schwann cells (in the peripheral nervous system) or ... Although glial cells are not involved with the transmission of electrical signals, they communicate and provide important ... certain neuronal axons are covered with myelin sheaths. Myelin is a multilamellar membrane that enwraps the axon in segments ... To be specific, myelin wraps multiple times around the axonal segment, forming a thick fatty layer that prevents ions from ...
Myelin proteins make up the myelin sheath that functions in protecting nerve axons, maintaining structural integrity, and ... apoptosis which will prevent the death of healthy cells and prevent gliosis which will prevent the formation of a glial scar. ... In patients with MS, autoreactive lymphocytes such as T and B cells cause damage to the CNS by attacking myelin proteins. ... They can also stimulate local progenitor cells to produce replacement cells that can assist in rebuilding the myelin sheath. ...
They are named glial scars because they are produced by glial cells, mainly astrocytes, and their presence prevents ... The oligodendrocytes that originally formed a myelin sheath cannot completely rebuild a destroyed myelin sheath. However, the ... myelin stains (Luxol fast blue/periodic acid-Schiff, Luxol fast blue/hematoxylin/eosin, or immunohistochemistry for myelin ... It seems to be a reduction of myelin phospholipids that correlates with a reduction of the myelin water fraction. The DAWM ...
Nielsen AL, Holm IE, Johansen M, Bonven B, Jørgensen P, Jørgensen AL (August 2002). "A new splice variant of glial fibrillary ... Myelin. *Myelin basic protein *PMP2. *Myelin proteolipid protein *PLP1. *Myelin oligodendrocyte glycoprotein ...
keywords = "Ataxia, Cerebellum, Myelin, Paranodes",. author = "Garcia-Fresco, {German P.} and Sousa, {Aurea D.} and Pillai, { ... Axo-glial junctions (AGJs) play a critical role in the organization and maintenance of molecular domains in myelinated axons. ... N2 - Axo-glial junctions (AGJs) play a critical role in the organization and maintenance of molecular domains in myelinated ... AB - Axo-glial junctions (AGJs) play a critical role in the organization and maintenance of molecular domains in myelinated ...
Bosio A, Binczek E, Stoffel W: Functional breakdown of the lipid bilayer of the myelin membrane in central and peripheral ... the pathogenesis of this lysosomal storage disorder beside its glial certainly also has a neuronal component. For the spiral ... Beside lysosomal storage of sulfatide in myelin forming glia cells, examination of ASA-deficient mice has clearly shown, that ... Bosio A, Binczek E, Haupt WF, Stoffel W: Composition and biophysical properties of myelin lipid define the neurological defects ...
Transplantation of glial cells enhances action potential conduction of amyelinated spinal cord axons in the myelin-deficient ... Transplantation of glial cells enhances action potential conduction of amyelinated spinal cord axons in the myelin-deficient ... Transplantation of glial cells enhances action potential conduction of amyelinated spinal cord axons in the myelin-deficient ... Transplantation of glial cells enhances action potential conduction of amyelinated spinal cord axons in the myelin-deficient ...
... the specimen was immunofluorescently labeled with mouse anti-myelin BP and rabbit anti-GFAP primary antibodies followed by goat ... In order to visualize myelin sheaths and astroglia in a rat brain sagittal tissue section (shown above), ... Distribution of Myelin Basic Protein and Glial Fibrillary Acidic Protein in Rat Brain Tissue. In order to visualize myelin ... Distribution of Myelin Basic Protein and Glial Fibrillary Acidic Protein in Rat Brain Tissue ...
Relation of C-6 glial cells in culture to myelin. J J Volpe, K Fujimoto, J C Marasa, H C Agrawal ... Relation of C-6 glial cells in culture to myelin Message Subject (Your Name) has forwarded a page to you from Biochemical ... C-6 glial cells were shown to contain enriched in their plasma membranes an enzyme, 2′:3′-cyclic nucleotide 3′-phosphohydrolase ... The data indicate that C-6 cells exhibit features of myelin-producing glia as well as astrocytes. ...
... in glial cells and myelin in the mammalian central nervous system. Those... ... the evidence for CA in myelin and in glial cells of both types will be discussed. Possible functions for the CA in myelin and ... White Matter Glial Cell Gray Matter Glial Fibrillary Acidic Protein Carbonic Anhydrase These keywords were added by machine and ... Cammer W. (1991) Carbonic Anhydrase in Myelin and Glial Cells in the Mammalian Central Nervous System. In: Dodgson S.J., ...
These glial cells are responsible for the formation of myelin sheaths around axons within the central nervous system: ...
Advisory Committee for Pilot Glial/Myelin Research (.pdf). National MS Society Scientific Peer Review Committee for Pilot ... Fast Forward and Myelin Repair Foundation (Start Up, Apr. 2012) (.pdf). Despite the availability of new oral medicines, broader ...
Finally, we examine evidence that myelin plasticity may be subject to critical periods. Taken together, the present review aims ... emphasizing the bidirectional interplay between neurons and myelinating glial cells to better inform future investigations of ... Finally, we examine evidence that myelin plasticity may be subject to critical periods. Taken together, the present review aims ... emphasizing the bidirectional interplay between neurons and myelinating glial cells to better inform future investigations of ...
... called glial cells, in invertebrates.[14][15] These glial wraps are quite different from vertebrate compact myelin, formed, as ... The Myelin Project, project to regenerate myelin. *Myelin Repair Foundation, a nonprofit medical research foundation for ... which is the most abundant protein in CNS myelin, but only a minor component of PNS myelin. In the PNS, myelin protein zero ( ... Myelin is formed in the central nervous system (CNS; brain, spinal cord and optic nerve) by glial cells called oligodendrocytes ...
It also features new techniques for imaging myelin changes in the brain and for screening molecular targets, as well as ... Human glial progenitor cell-based treatment and modeling of myelin disease. Goldman SA, Nedergaard M, Windrem MS. Glial ... Human Glial Progenitor Cell-based Treatment and Modeling of Myelin Disease. Steven A. Goldman (University of Rochester Medical ... Glial progenitor cell transplantation could restore myelin in people with progressive MS.*Neural stem cell transplantation ...
Myelin proteolipid protein (PLP), the major myelin protein in the CNS, has been thought to function in myelin assembly. Thus, ... N2 - Myelin proteolipid protein (PLP), the major myelin protein in the CNS, has been thought to function in myelin assembly. ... AB - Myelin proteolipid protein (PLP), the major myelin protein in the CNS, has been thought to function in myelin assembly. ... abstract = "Myelin proteolipid protein (PLP), the major myelin protein in the CNS, has been thought to function in myelin ...
The glial cells express a loss of myelin. This loss of myelin is different from that seen in other diseases where ... In affected cells, the protein is reduced to about 50%, which is acceptable for functionality in most cells, but not in glial ... A unique characteristic of VWM is that only oligodendrocytes and astrocytes are negatively affected while other glial cells and ... Melatonin has been shown to provide cytoprotective traits to glial cells exposed to stressors such as excitotoxicity and ...
Glial-specific proteins were identified by using the Schwann cell myelin protein (SMP) mAb (30, 31) and 1E8 mAb that recognizes ... Activation of Myelin Genes during Transdifferentiation from Melanoma to Glial Cell Phenotype ... 2 H and I). Staining with antibodies recognizing glial Po and SMP proteins showed that both glial markers are expressed by a ... glial cells and neurons (GN progenitors), glial cells, melanocytes, and neurons (GMN progenitors). In addition, the NC ...
Myelin basic protein (MBP) binds to negatively charged lipids on the cytosolic surface of oligodendrocyte membranes and is ... responsible for adhesion of these surfaces in the multilayered myelin... ... A combinatorial network of evolutionarily conserved myelin basic protein regulatory sequences confers distinct glial-specific ... Moscarello MA (1997) Myelin basic protein, the "executive" molecule of the myelin membrane. In: Juurlink BHJ, Devon RM, ...
It is an outgrowth of a type of glial cell. The production of the myelin sheath is called myelination or myelinogenesis. In ... The Myelin Project, project to regenerate myelin Myelin Repair Foundation, a nonprofit medical research foundation for multiple ... The Myelin Project Athabasca University Biological Psychology Website The MS Information Sourcebook, Myelin The Myelin Repair ... Myelin basic protein (MBP) constitutes ~23% of myelin protein, myelin oligodendrocyte glycoprotein, and proteolipid protein ( ...
Neurodegeneration, neuroprotection, glial cells, and myelin in multiple sclerosis. Publication Date: March 2009 ...
So all these demyelinating diseases will affect either central myelin or peripheral myelin. ... Glial Cells. To view this video please enable JavaScript, and consider upgrading to a web browser that supports HTML5 video ...
The 2011 Gordon Research Conference on Glial Biology: Functional Interactions Among Glia and Neurons will be held in Ventura, ... Myelin May 17 - 22, 2020 Neural Development August 9 - 14, 2020 Neuroimmune Communication in Health and Disease January 24 - 29 ... The broad and long-term goal of the conference is to increase our understanding of the manner in which glial cells interact ... Leaders in the field will discuss critical areas of glial research.. The program will have nine sessions that broadly address ...
... is an outgrowth of a glial cell. The production of the myelin sheath is called myelination. The production of myelin ... Some of the proteins that make up myelin are myelin basic protein (MBP), myelin oligodendrocyte glycoprotein (MOG), and ... Function of myelin layer. The main purpose of a myelin layer (or sheath) is to increase the speed at which impulses propagate ... Myelin is a dielectric (electrically insulating) material that forms a layer, the myelin sheath, usually around only the axon ...
... characterized by the expression of myelin genes and the elaboration of a multilayered myelin sheath (Lemke, 1988). ... Sox10, a Novel Transcriptional Modulator in Glial Cells. Kirsten Kuhlbrodt, Beate Herbarth, Elisabeth Sock, Irm Hermans- ... Sox10, a Novel Transcriptional Modulator in Glial Cells. Kirsten Kuhlbrodt, Beate Herbarth, Elisabeth Sock, Irm Hermans- ... Glial cells and their development have been studied extensively both in the CNS and in the PNS (for review, see Pfeiffer et al ...
... myelin sheath- wrapping around nerve fibers that insulate, protect, and support the neuron, nerve-bundle of axons ... Glial Cells- act as neural assistantsby providing structural support (entourage), ... 8. Glial Cells- act as neural assistantsby providing structural support (entourage). 8.1. Oligodentrocytes- cells that produce ... 9. myelin sheath- wrapping around nerve fibers that insulate, protect, and support the neuron. 10. nerve-bundle of axons. 10.1 ...
Here we will provide a general overview on the possible involvement of mGlu receptors expressed on glial cells in the ... Here we will provide a general overview on the possible involvement of mGlu receptors expressed on glial cells in the ... The expression of mGlu receptors has been also reported on oligodendrocytes, a glial cell type specifically involved in the ... The expression of mGlu receptors has been also reported on oligodendrocytes, a glial cell type specifically involved in the ...
Keywords: myelin; glial scar; trophic factors; scaffolds; regeneration Figure 1. Diagram of the peripheral nervous system ... Within the myelin debris are proteins that inhibit regeneration, including Nogo proteins and myelin‐associated glycoprotein ( ... The glial scar in the CNS presents a highly inhibitory and disorganised terrain to regenerating neurites. ... Schnell L and Schwab ME (1990) Axonal regeneration in the rat spinal cord produced by an antibody against myelin‐associated ...
myelin. Supporting and protective cells of the ner-vous system; glial cells.. neuroglia. ...
  • Mice deficient in either the axonal protein NCP1 or the glial ceramide galactosyltransferase (CGT) display disruptions in AGJs and severe ataxia. (elsevier.com)
  • Neurexin IV/Caspr1/paranodin (NCP1) is an important player in the formation of AGJs because it recruits a paranodal complex implicated in the tethering of glial proteins to the axonal membrane and cytoskeleton. (elsevier.com)
  • Axo-glial junctions (AGJs) play a critical role in the organization and maintenance of molecular domains in myelinated axons. (elsevier.com)
  • Myelin is a dielectric ( electrically insulating ) material that forms a layer, the myelin sheath , usually around only the axon of a neuron . (princeton.edu)
  • Although evidence was mounting of glial-related abnormalities in schizophrenia prior to the new study, researchers didn't know whether these might just be secondary to a neuron-rooted illness process. (nih.gov)
  • As a result, shiverer mice have little or no compact CNS myelin. (jneurosci.org)
  • Studying transgenic shiverer mice permits distinctions between parameters affected by the presence of compact myelin and those requiring a full complement of compact myelin. (jneurosci.org)
  • Young adult Plp1- null mice exhibited subtle but substantial behavioral alterations, indicative of an early impact of mild myelin disruption. (elifesciences.org)
  • The phenomenon can be seen in myelin lesions from individuals with MS and in mice with a similar condition. (fiercebiotech.com)
  • The main purpose of a myelin sheath is to increase the speed at which impulses propagate along the myelinated fiber. (wikipedia.org)
  • Myelin is considered a defining characteristic of the (gnathostome) vertebrates , but it has also arisen by parallel evolution in some invertebrates. (princeton.edu)
  • The discontinuous structure of the myelin sheath results in saltatory conduction , whereby the action potential "jumps" from one node of Ranvier, over a long myelinated stretch of the axon called the internode, before "recharging" at the next node of Ranvier, and so on, until it reaches the axon terminal . (wikipedia.org)
  • Although the function of myelin in facilitating the efficient and rapid propagation of nerve impulses by saltatory conduction has long been known, the basic mechanisms that drive the extension and wrapping of the glial membrane around the axon remain poorly understood. (rupress.org)
  • SCoRe images captured in vivo at the indicated ages show marked, age-dependent changes in myelin density in layer I of the somatosensory cortex. (scitechdaily.com)
  • The production of myelin occurs in the fourteenth week of fetal development, while very little amounts of myelin exist in the brain at the time of birth. (princeton.edu)
  • In humans, the production of myelin begins in the fourteenth week of fetal development, although little myelin exists in the brain at the time of birth. (phys.org)
  • Although it is known that transplantation of neural tissue can lead to functional improvement in models of neurological disease characterized by neuronal loss, less is known about results of transplantation in disorders of myelin. (pnas.org)
  • We discuss how thyroid hormone affects glial cell function followed by an overview on the fine-tuned regulation of T3 generation by D2 in different glial subtypes. (hindawi.com)
  • Here, in a rat model of osmotic demyelination syndrome, we found that massive astrocyte death occurred after rapid correction of hyponatremia, delineating the regions of future myelin loss. (asnjournals.org)
  • The internodes are the myelin segments and the gaps between are referred to as nodes. (wikipedia.org)
  • Vesicles and other organelles are also increased at the nodes, which suggest that there is a bottleneck of axonal transport in both directions as well as local axonal-glial signaling. (wikipedia.org)