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 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)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.Mice, Quaking: Mice homozygous for the mutant autosomal recessive gene, quaking (qk), associated with disorder in myelin formation and manifested by axial tremors.N-Acylsphingosine Galactosyltransferase: An enzyme that catalyzes the conversion of UDP-galactose and N-acylsphingosine to D-galactosylceramide and UDP.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)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.Schwann Cells: Neuroglial cells of the peripheral nervous system which form the insulating myelin sheaths of peripheral axons.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.Mice, Neurologic Mutants: Mice which carry mutant genes for neurologic defects or abnormalities.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.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.Demyelinating Diseases: Diseases characterized by loss or dysfunction of myelin in the central or peripheral nervous system.Axons: Nerve fibers that are capable of rapidly conducting impulses away from the neuron cell body.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.Central Nervous System: The main information-processing organs of the nervous system, consisting of the brain, spinal cord, and meninges.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.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.Compact Disks: Computer disks storing data with a maximum reduction of space and bandwidth. The compact size reduces cost of transmission and storage.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)Nerve 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.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.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.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)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.Spinal Cord: A cylindrical column of tissue that lies within the vertebral canal. It is composed of WHITE MATTER and GRAY MATTER.Sulfoglycosphingolipids: GLYCOSPHINGOLIPIDS with a sulfate group esterified to one of the sugar groups.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.Models, Molecular: Models used experimentally or theoretically to study molecular shape, electronic properties, or interactions; includes analogous molecules, computer-generated graphics, and mechanical structures.Protein Conformation: The characteristic 3-dimensional shape of a protein, including the secondary, supersecondary (motifs), tertiary (domains) and quaternary structure of the peptide chain. PROTEIN STRUCTURE, QUATERNARY describes the conformation assumed by multimeric proteins (aggregates of more than one polypeptide chain).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.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.-.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.Mice, Jimpy: Myelin-deficient mutants which are from the inbred Tabby-Jimpy strain.Protein Folding: Processes involved in the formation of TERTIARY PROTEIN STRUCTURE.Charcot-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)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)Cuprizone: Copper chelator that inhibits monoamine oxidase and causes liver and brain damage.Nerve Regeneration: Renewal or physiological repair of damaged nerve tissue.Ganglioside Galactosyltransferase: Catalyzes the final step in the galactocerebroside biosynthesis pathway.Peptide Fragments: Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.Protein Structure, Secondary: The level of protein structure in which regular hydrogen-bond interactions within contiguous stretches of polypeptide chain give rise to alpha helices, beta strands (which align to form beta sheets) or other types of coils. This is the first folding level of protein conformation.Protein Structure, Tertiary: The level of protein structure in which combinations of secondary protein structures (alpha helices, beta sheets, loop regions, and motifs) pack together to form folded shapes called domains. Disulfide bridges between cysteines in two different parts of the polypeptide chain along with other interactions between the chains play a role in the formation and stabilization of tertiary structure. Small proteins usually consist of only one domain but larger proteins may contain a number of domains connected by segments of polypeptide chain which lack regular secondary structure.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.Lenses: Pieces of glass or other transparent materials used for magnification or increased visual acuity.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.Circular Dichroism: A change from planar to elliptic polarization when an initially plane-polarized light wave traverses an optically active medium. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)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.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.X-Ray Diffraction: The scattering of x-rays by matter, especially crystals, with accompanying variation in intensity due to interference effects. Analysis of the crystal structure of materials is performed by passing x-rays through them and registering the diffraction image of the rays (CRYSTALLOGRAPHY, X-RAY). (From McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)Nerve Tissue ProteinsFishes, PoisonousScattering, Small Angle: Scattering of a beam of electromagnetic or acoustic RADIATION, or particles, at small angles by particles or cavities whose dimensions are many times as large as the wavelength of the radiation or the de Broglie wavelength of the scattered particles. Also know as low angle scattering. (McGraw-Hill Dictionary of Scientific and Technical Terms, 6th ed) Small angle scattering (SAS) techniques, small angle neutron (SANS), X-ray (SAXS), and light (SALS, or just LS) scattering, are used to characterize objects on a nanoscale.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.Rats, Inbred LewEarly 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.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.Mice, Inbred C57BLBase Sequence: The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.Protein Denaturation: Disruption of the non-covalent bonds and/or disulfide bonds responsible for maintaining the three-dimensional shape and activity of the native protein.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.Mice, Transgenic: Laboratory mice that have been produced from a genetically manipulated EGG or EMBRYO, MAMMALIAN.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.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)Miniaturization: The design or construction of objects greatly reduced in scale.Equipment Design: Methods of creating machines and devices.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.Holography: The recording of images in three-dimensional form on a photographic film by exposing it to a laser beam reflected from the object under study.Crystallography, X-Ray: The study of crystal structure using X-RAY DIFFRACTION techniques. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)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.
  • Compact myelin dictates the differential targeting of two sodium channel isoforms in the same axon. (nih.gov)
  • Together, these data show that sodium channel isoforms are differentially targeted to distinct domains of the same axon in a process associated with formation of compact myelin. (nih.gov)
  • however, there are distinct differences in the makeup of the myelin in the CNS and PNS (Table 1 ), and only the CNS myelin is damaged in MS. In addition, Schwann cells only myelinate a single segment of an axon, whereas each individual oligodendrocyte can myelinate numerous separate segments of axons in the CNS (Figure 1(a) ). (hindawi.com)
  • Altered axon-Schwann cell interactions in PNS myelin-deficient Trembler mice result in changed axonal transport rates, neurofilament and microtubule-associated protein phosphorylation, neurofilament density, and microtubule stability. (jneurosci.org)
  • Although proteins responsible for compaction of CNS and PNS myelin are well characterized, less is known about proteins mediating axon-glial cell interactions in either CNS or PNS. (jneurosci.org)
  • Because axon degeneration appear to start rather early with respect to myelin degenerations, these lipids might be required for glial support of axon function. (jneurosci.org)
  • The internodal glial membranes are fused to form compact myelin , whereas the cytoplasm-filled paranodal loops of myelinating cells are spirally wrapped around the axon at both sides of the nodes. (wikipedia.org)
  • Schwann Cell O-GlcNAc Glycosylation Is Required for Myelin Maintenance and Axon Integrity. (nih.gov)
  • Myelin is a lipid -rich (fatty) substance that surrounds nerve cell axons (the nervous system's "wires") to insulate them and increase the rate at which electrical impulses (called action potentials ) are passed along the axon. (wikipedia.org)
  • The myelinated axon can be likened to an electrical wire (the axon) with insulating material (myelin) around it. (wikipedia.org)
  • These glial wraps are quite different from vertebrate compact myelin, formed, as indicated above, by concentric wrapping of the myelinating cell process multiple times around the axon. (wikipedia.org)
  • A ) Normal myelin with compacted (dark blue) and uncompacted (light blue) channels form a multilamellar structure around the axon. (jci.org)
  • All these models show a clear distinction between the ability of myelin to support axonal conductance and its ability to provide trophic support to the axon. (jci.org)
  • The formation of myelin, which involves reciprocal signaling between neurons and glial cells, a dramatic morphological transformation of the glial cells, and organization of the axon into different specialized domains, is fascinating but nonetheless poorly understood. (stanford.edu)
  • From these images, a three-pool model was used to estimate the fraction of water in myelin, myelinated axon, and mixed water compartments (or pools) in six WM regions. (ajnr.org)
  • Cell-culture studies have identified molecular mechanisms regulating myelination by electrical activity, and myelin also limits the critical period for learning through inhibitory proteins that suppress axon sprouting and synaptogenesis. (pubmedcentralcanada.ca)
  • We used rodent sensory neuron and SC cultures in which initiation of myelinogenesis is relatively synchronized and bright field conditions that allowed resolution of the axon, compact myelin, and position of the SC nucleus. (rupress.org)
  • The segmental arrangement of myelin along the axon maximizes the speed and efficiency of saltatory conduction. (healio.com)
  • Myelin suppresses axon regeneration by PIR-B/SHP-mediated inhibition of Trk activity. (genes2cognition.org)
  • When nerve cells are unmyelinated, the axon has a smaller diameter and contains neurofilaments that are less modified and are more compact. (redorbit.com)
  • Repetitive stimulation of a fibre is known to result in accumulation of extracellular potassium ions, especially between the axon and the myelin. (royalsocietypublishing.org)
  • In order to further understand the dynamic interaction that takes place between the myelin and the axon, we have modelled submyelin potassium accumulation and related changes in myelin resistance during prolonged high-frequency stimulation. (royalsocietypublishing.org)
  • In Shiverer mice, which lack compact myelin, Na(v)1.2 is found throughout adult axons, whereas little Na(v)1.6 is detected. (nih.gov)
  • a) Myelin is produced by the oligodendrocyte (OLIG) and is laid down in segments along nerve axons. (hindawi.com)
  • To determine whether PNS and CNS myelination have equivalent effects on axons, neurofilaments, and microtubules in CNS, myelin-deficient shiverer axons were examined. (jneurosci.org)
  • In both CNS and PNS, myelination is a complex process in which glial cells extend processes that enwrap axons and generate compact myelin ( Morell and Quarles, 1999 ). (jneurosci.org)
  • To investigate whether PNS and CNS myelination affects axons equivalently, we examined CNS myelin-deficient shiverer mice. (jneurosci.org)
  • Myelin is the lipid-rich insulatory material that ensheathes axons in the central nervous system, increasing the speed of signal conduction and providing precise temporal control over the propagation of action potentials within brain networks. (frontiersin.org)
  • Myelin is considered a defining characteristic of the jawed vertebrates ( gnathostomes ), though axons are ensheathed by a type of cell, called glial cells, in invertebrates. (wikipedia.org)
  • B ) In PLP-null animals, myelin appears to be ultrastructurally normal, but the underlying axons show signs of degeneration. (jci.org)
  • These cells were shown to express myelin basic protein, associate with axons, and support nodal architecture as well as display proper compact, multilayer myelination in electron microscopic analysis. (wingsforlife.com)
  • Myelin is the fatty tissue that wraps around nerve fibers, or axons, in the nervous system. (medicalxpress.com)
  • Three cell adhesion molecules, contactin-1 (CNTN1) and contactin-associated protein-1 (CASPR1) in the axons, and neurofascin155 (NF155) in the myelin are responsible for the adhesion and the formation of septate-like junctions (also known as transverse bands) (FIG. 1). (docme.ru)
  • Main pathological features in sALS are loss of myelin and axons in the pyramidal tracts and anterior spinal roots, chromatolysis of motor neurons, axonal spheroids in the anterior horn, cystatin C-containing Bunina bodies in motor neurons, ubiquitin-immunoreactive TDP-43-positive skein-like and spherical inclusions in motor neurons, and TDP-43 inclusions in oligodendroglial cells. (aging-us.com)
  • During myelination, a layer of myelin (electrically insulating material) wraps or forms around the axons (part of the nerve cell that conducts electrical impulses). (redorbit.com)
  • The encoded protein contains a large hydrophobic extracellular domain and a smaller basic intracellular domain, which are essential for the formation and stabilization of the multilamellar structure of the compact myelin. (origene.com)
  • Changes in myelin structure mediated by high extracellular potassium are modelled as a concentration-dependent decrease in myelin resistance. (royalsocietypublishing.org)
  • The model exhibits oscillatory, chaotic or stationary behaviour with respect to extracellular potassium concentration and myelin resistance and the fibre therefore shows periodic, chaotic or complete excitation block at high frequencies of stimulation. (royalsocietypublishing.org)
  • The genetic defect in shiverer is a deletion in the myelin basic protein (MBP) gene, an essential component of CNS myelin. (jneurosci.org)
  • Shiverer mice contain a deletion in the MBP gene, do not produce MBP protein, and have no compact CNS myelin (for review, see Readhead and Hood, 1990 ). (jneurosci.org)
  • Growth arrest-specific protein 3 (GAS-3), also called peripheral myelin protein 22 (PMP22), is a protein which in humans is encoded by the PMP22 gene. (wikipedia.org)
  • We first demonstrated that loss of function mutations in the MTMR2 (Myotubularin-related 2) gene cause autosomal recessive demyelinating Charcot-Marie-Tooth type 4B1 (CMT4B1, OMIM #601382) neuropathy with myelin outfoldings . (prolekare.cz)
  • Researchers also knew that a particular mutation in that gene caused a neurological defect known as hypomyelinating leukodystrophy, which creates a myelin deficit in the brain, leading to the deterioration of both motor skills and mental acuity. (medicalxpress.com)
  • We report a case of co-existence of SBMA and atypical HNPP with genetic confirmation of CAG expansion in the androgen receptor (AR) gene and deletion of the peripheral myelin protein 22 (PMP22) gene. (readbyqxmd.com)
  • Point mutations in the peripheral myelin protein 22 (PMP22) gene have been identified to cause demyelinating Charcot-Marie-Tooth disease (CMT) and hereditary neuropathy with liability to pressure palsy (HNPP). (readbyqxmd.com)
  • An important gene associated with Central Pontine Myelinolysis is MBP (Myelin Basic Protein), and among its related pathways/superpathways are Sudden Infant Death Syndrome (SIDS) Susceptibility Pathways and Aquaporin-mediated transport . (malacards.org)
  • and c. identify for the first time extensive gene up-regulation of neurotransmission and synaptic-related genes, together with significant down-regulation of oligodendrocyte- and myelin-related genes, as important contributors to the pathogenesis of frontal cortex alterations in the sALS/frontotemporal lobar degeneration spectrum complex at stages with no apparent cognitive impairment. (aging-us.com)
  • One theory proposes that in regions of compact interdigitation of white and gray matter, cellular edema, which is caused by fluctuating osmotic forces, results in compression of fiber tracts and induces demyelination. (medscape.com)
  • The ENU20884 mutants exhibit demyelination and aberrant myelin structures, as well as severe electrophysiological abnormalities seen by a reduction in sciatic nerve conduction velocity and compound muscle action potentials. (jax.org)
  • 2017) demonstrated detachment of terminal myelin loops from the axolemma without classical macrophage-mediated demyelination antibodies in 1 patient with CNTN1 antibodies. (docme.ru)
  • Immunization with myelin-associated antigens including MBP significantly promotes recovery after spinal cord contusion injury in the rat model. (biosensis.com)
  • Divalent Cations Induce a Compaction of Intrinsically Disordered Myelin Basic Protein Biochemical and Biophysical Research Communications. (jove.com)
  • Here, we show by nanopore analysis that the divalent cations copper and zinc induce a compaction of the extended protein in vitro, suggestive of a tertiary conformation that may reflect its arrangement in myelin. (jove.com)
  • The basic protein of myelin is an extrinsic component and must be localised at the surface of the lipid phase at either the external or cytoplasmic face of the lamellae. (springer.com)
  • The results presented in this study improve our understanding and allows for better interpretation of MWF in the presence of myelin debris. (ovid.com)
  • One characteristic of white matter that may contribute to its susceptibility to anoxic injury is the presence of myelin. (docme.ru)
  • This appearance is believed to be the result of the lack of formation of myelin (hypomyelination or dysmyelination) ( 6 ). (ajnr.org)
  • Through a mechanism that is only partly understood, the shift in water and brain molecules leads to the destruction of myelin, a substance that surrounds and protects nerve fibers. (malacards.org)
  • In mature myelin, the predominant splice isoform of classic MBP is 18.5kDa. (jove.com)
  • Understanding the molecular basis for myelin formation is essential with regard to various neurological illnesses that involve a loss of the protective myelin layer. (bio-medicine.org)
  • This distinction is critical since the Hu lab saw that TMEM106B is expressed in the cells that are responsible for forming the protective myelin tissue. (medicalxpress.com)
  • The study, "Phosphorylation of Highly Conserved Neurofilament-M KSP Repeats Is Not Required for Myelin-Dependent Radial Axonal Growth," was published in The Journal of Neuroscience. (redorbit.com)