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-specific proteins that play a structural or regulatory role in the genesis and maintenance of the lamellar MYELIN SHEATH structure.
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)
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 homozygous for the mutant autosomal recessive gene, quaking (qk), associated with disorder in myelin formation and manifested by axial tremors.
An enzyme that catalyzes the conversion of UDP-galactose and N-acylsphingosine to D-galactosylceramide and UDP.
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)
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.
Neuroglial cells of the peripheral nervous system which form the insulating myelin sheaths of peripheral axons.
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 which carry mutant genes for neurologic defects or abnormalities.
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.
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.
Diseases characterized by loss or dysfunction of myelin in the central or peripheral nervous system.
Nerve fibers that are capable of rapidly conducting impulses away from the neuron cell body.
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.
The main information-processing organs of the nervous system, consisting of the brain, spinal cord, and meninges.
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.
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.
Computer disks storing data with a maximum reduction of space and bandwidth. The compact size reduces cost of transmission and storage.
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)
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.
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)
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.
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.
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)
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.
A cylindrical column of tissue that lies within the vertebral canal. It is composed of WHITE MATTER and GRAY MATTER.
GLYCOSPHINGOLIPIDS with a sulfate group esterified to one of the sugar groups.
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 used experimentally or theoretically to study molecular shape, electronic properties, or interactions; includes analogous molecules, computer-generated graphics, and mechanical structures.
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).
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.
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.-.
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.
Myelin-deficient mutants which are from the inbred Tabby-Jimpy strain.
Processes involved in the formation of TERTIARY PROTEIN STRUCTURE.
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)
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)
Copper chelator that inhibits monoamine oxidase and causes liver and brain damage.
Renewal or physiological repair of damaged nerve tissue.
Catalyzes the final step in the galactocerebroside biosynthesis pathway.
Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.
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.
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.
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.
Pieces of glass or other transparent materials used for magnification or increased visual acuity.
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.
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)
Electron microscopy in which the ELECTRONS or their reaction products that pass down through the specimen are imaged below the plane of the specimen.
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.
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 proteins' are specialized proteins found within the nervous system's biological tissue, including neurofilaments, neuronal cytoskeletal proteins, and neural cell adhesion molecules, which facilitate structural support, intracellular communication, and synaptic connectivity essential for proper neurological function.
'Poisonous fishes' are aquatic organisms belonging to the Phylum Chordata and Class Pisces, that contain toxic substances either in their tissues or secretions, which can cause harmful or lethal effects when ingested, touched, or coming into contact with their released toxins.
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 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 Lew' is a strain of laboratory rat that is widely used in biomedical research, known for its consistent genetic background and susceptibility to certain diseases, which makes it an ideal model for studying the genetic basis of complex traits and disease processes.
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.
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.
Inbred C57BL mice are a strain of laboratory mice that have been produced by many generations of brother-sister matings, resulting in a high degree of genetic uniformity and homozygosity, making them widely used for biomedical research, including studies on genetics, immunology, cancer, and neuroscience.
The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.
Disruption of the non-covalent bonds and/or disulfide bonds responsible for maintaining the three-dimensional shape and activity of the native protein.
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.
Laboratory mice that have been produced from a genetically manipulated EGG or EMBRYO, MAMMALIAN.
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.
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)
The design or construction of objects greatly reduced in scale.
Methods of creating machines and devices.
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.
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.
The study of crystal structure using X-RAY DIFFRACTION techniques. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
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.

The myelin sheath is a multilayered, fatty substance that surrounds and insulates many nerve fibers in the nervous system. It is essential for the rapid transmission of electrical signals, or nerve impulses, along these nerve fibers, allowing for efficient communication between different parts of the body. The myelin sheath is produced by specialized cells called oligodendrocytes in the central nervous system (CNS) and Schwann cells in the peripheral nervous system (PNS). Damage to the myelin sheath, as seen in conditions like multiple sclerosis, can significantly impair nerve function and result in various neurological symptoms.

Myelin proteins are proteins that are found in the myelin sheath, which is a fatty (lipid-rich) substance that surrounds and insulates nerve fibers (axons) in the nervous system. The myelin sheath enables the rapid transmission of electrical signals (nerve impulses) along the axons, allowing for efficient communication between different parts of the nervous system.

There are several types of myelin proteins, including:

1. Proteolipid protein (PLP): This is the most abundant protein in the myelin sheath and plays a crucial role in maintaining the structure and function of the myelin sheath.
2. Myelin basic protein (MBP): This protein is also found in the myelin sheath and helps to stabilize the compact structure of the myelin sheath.
3. Myelin-associated glycoprotein (MAG): This protein is involved in the adhesion of the myelin sheath to the axon and helps to maintain the integrity of the myelin sheath.
4. 2'3'-cyclic nucleotide 3' phosphodiesterase (CNP): This protein is found in oligodendrocytes, which are the cells that produce the myelin sheath in the central nervous system. CNP plays a role in maintaining the structure and function of the oligodendrocytes.

Damage to myelin proteins can lead to demyelination, which is a characteristic feature of several neurological disorders, including multiple sclerosis (MS), Guillain-Barré syndrome, and Charcot-Marie-Tooth disease.

Myelin P0 protein, also known as P0 or MPZ (myelin protein zero), is a major structural component of the myelin sheath in the peripheral nervous system. The myelin sheath is a multilayered membrane that surrounds and insulates nerve fibers to increase the speed of electrical impulse transmission.

P0 protein is a transmembrane glycoprotein, which means it spans the lipid bilayer of the myelin membrane and has sugar molecules (glycans) attached to it. It plays a crucial role in maintaining the compact structure of the myelin sheath by forming homodimers that interact with each other through their extracellular domains, creating tight junctions between the apposing layers of the myelin membrane.

P0 protein also contributes to the stability and integrity of the myelin sheath by interacting with other myelin proteins, such as connexin 32 and peripheral myelin protein 22 (PMP22). Mutations in the MPZ gene can lead to various peripheral neuropathies, including Charcot-Marie-Tooth disease type 1B and Dejerine-Sottas syndrome.

Myelin Basic Protein (MBP) is a key structural protein found in the myelin sheath, which is a multilayered membrane that surrounds and insulates nerve fibers (axons) in the nervous system. The myelin sheath enables efficient and rapid transmission of electrical signals (nerve impulses) along the axons, allowing for proper communication between different neurons.

MBP is one of several proteins responsible for maintaining the structural integrity and organization of the myelin sheath. It is a basic protein, meaning it has a high isoelectric point due to its abundance of positively charged amino acids. MBP is primarily located in the intraperiod line of the compact myelin, which is a region where the extracellular leaflets of the apposing membranes come into close contact without fusing.

MBP plays crucial roles in the formation, maintenance, and repair of the myelin sheath:

1. During development, MBP helps mediate the compaction of the myelin sheath by interacting with other proteins and lipids in the membrane.
2. MBP contributes to the stability and resilience of the myelin sheath by forming strong ionic bonds with negatively charged phospholipids in the membrane.
3. In response to injury or disease, MBP can be cleaved into smaller peptides that act as chemoattractants for immune cells, initiating the process of remyelination and repair.

Dysregulation or damage to MBP has been implicated in several demyelinating diseases, such as multiple sclerosis (MS), where the immune system mistakenly attacks the myelin sheath, leading to its degradation and loss. The presence of autoantibodies against MBP is a common feature in MS patients, suggesting that an abnormal immune response to this protein may contribute to the pathogenesis of the disease.

I'm not aware of a medical condition known as "Quaking Mice." However, "quaking" is a phenotype observed in laboratory mice that are used as models for certain genetic disorders.

The "quaking" phenotype is caused by a mutation in the QKI gene, which plays a crucial role in the development and function of the nervous system. Mice with this mutation have abnormal myelination (the process of forming a protective sheath around nerve fibers) in their central nervous system, leading to tremors, ataxia (loss of coordination), and other neurological symptoms.

The Quaking mouse model is often used in research to study the genetic and molecular mechanisms underlying demyelinating disorders, such as multiple sclerosis, and to test potential therapies for these conditions.

N-Acylsphingosine Galactosyltransferase is a type of enzyme that plays a role in the synthesis of galactosylceramide, which is a critical component of the myelin sheath in the nervous system. The enzyme's systematic name is UDP-galactose:N-acylsphingosine galactosyltransferase, and it catalyzes the following chemical reaction:
UDP-galactose + N-acylsphingosine = UDP + D-galactosyl-N-acylsphingosine.
This enzyme is also known as galactosylceramide synthase, and it is involved in the biosynthesis of galactolipids, which are essential for the formation and maintenance of the myelin sheath around neurons. Deficiencies in this enzyme have been linked to certain genetic disorders, such as Krabbe disease and hereditary sensory and autonomic neuropathy type I.

Myelin Proteolipid Protein (PLP) is a major component of the myelin sheath, which is a fatty insulating substance that covers and protects nerve fibers in the central nervous system (CNS). PLP makes up about 50% of the proteins found in the myelin sheath. It plays a crucial role in the structure and function of the myelin sheath, including maintaining its compactness and stability. Defects or mutations in the gene that encodes for PLP can lead to various demyelinating diseases, such as X-linked adrenoleukodystrophy (X-ALD) and Pelizaeus-Merzbacher disease (PMD), which are characterized by the degeneration of the myelin sheath and subsequent neurological impairments.

Ranvier's nodes, also known as nodes of Ranvier, are specialized structures in the nervous system. They are gaps in the myelin sheath, a fatty insulating substance that surrounds the axons of many neurons, leaving them exposed. These nodes play a crucial role in the rapid transmission of electrical signals along the neuron. The unmyelinated sections of the axon at the nodes have a higher concentration of voltage-gated sodium channels, which generate the action potential that propagates along the neuron. The myelinated segments between the nodes, called internodes, help to speed up this process by allowing the action potential to "jump" from node to node, a mechanism known as saltatory conduction. This process significantly increases the speed of neural impulse transmission, making it more efficient. Ranvier's nodes are named after Louis-Antoine Ranvier, a French histologist and physiologist who first described them in the late 19th century.

Schwann cells, also known as neurolemmocytes, are a type of glial cell that form the myelin sheath around peripheral nervous system (PNS) axons, allowing for the rapid and efficient transmission of nerve impulses. These cells play a crucial role in the maintenance and function of the PNS.

Schwann cells originate from the neural crest during embryonic development and migrate to the developing nerves. They wrap around the axons in a spiral fashion, forming multiple layers of myelin, which insulates the nerve fibers and increases the speed of electrical impulse transmission. Each Schwann cell is responsible for myelinating a single segment of an axon, with the gaps between these segments called nodes of Ranvier.

Schwann cells also provide structural support to the neurons and contribute to the regeneration of injured peripheral nerves by helping to guide the regrowth of axons to their targets. Additionally, Schwann cells can participate in immune responses within the PNS, such as releasing cytokines and chemokines to recruit immune cells during injury or infection.

Oligodendroglia are a type of neuroglial cell found in the central nervous system (CNS) of vertebrates, including humans. These cells play a crucial role in providing support and insulation to nerve fibers (axons) in the CNS, which includes the brain and spinal cord.

More specifically, oligodendroglia produce a fatty substance called myelin that wraps around axons, forming myelin sheaths. This myelination process helps to increase the speed of electrical impulse transmission (nerve impulses) along the axons, allowing for efficient communication between different neurons.

In addition to their role in myelination, oligodendroglia also contribute to the overall health and maintenance of the CNS by providing essential nutrients and supporting factors to neurons. Dysfunction or damage to oligodendroglia has been implicated in various neurological disorders, such as multiple sclerosis (MS), where demyelination of axons leads to impaired nerve function and neurodegeneration.

Neurologic mutant mice are genetically engineered or spontaneously mutated rodents that are used as models to study various neurological disorders and conditions. These mice have specific genetic modifications or mutations that affect their nervous system, leading to phenotypes that resemble human neurological diseases.

Some examples of neurologic mutant mice include:

1. Alzheimer's disease models: Mice that overexpress genes associated with Alzheimer's disease, such as the amyloid precursor protein (APP) or presenilin 1 (PS1), to study the pathogenesis and potential treatments of this disorder.
2. Parkinson's disease models: Mice that have genetic mutations in genes associated with Parkinson's disease, such as alpha-synuclein or parkin, to investigate the mechanisms underlying this condition and develop new therapies.
3. Huntington's disease models: Mice that carry an expanded CAG repeat in the huntingtin gene to replicate the genetic defect seen in humans with Huntington's disease and study disease progression and treatment strategies.
4. Epilepsy models: Mice with genetic mutations that cause spontaneous seizures or increased susceptibility to seizures, used to investigate the underlying mechanisms of epilepsy and develop new treatments.
5. Stroke models: Mice that have surgical induction of stroke or genetic modifications that increase the risk of stroke, used to study the pathophysiology of stroke and identify potential therapeutic targets.

Neurologic mutant mice are essential tools in biomedical research, allowing scientists to investigate the complex interactions between genes and the environment that contribute to neurological disorders. These models help researchers better understand disease mechanisms, develop new therapies, and test their safety and efficacy before moving on to clinical trials in humans.

Myelin-Associated Glycoprotein (MAG) is a glycoprotein found on the surface of myelin sheaths, which are the protective insulating layers around nerve fibers in the nervous system. MAG plays a role in the adhesion and interaction between the myelin sheath and the axon it surrounds. It's particularly important during the development and maintenance of the nervous system. Additionally, MAG has been implicated in the regulation of neuronal growth and signal transmission. In certain autoimmune diseases like Guillain-Barré syndrome, the immune system may mistakenly attack MAG, leading to damage of the myelin sheath and associated neurological symptoms.

Peripheral nerves are nerve fibers that transmit signals between the central nervous system (CNS, consisting of the brain and spinal cord) and the rest of the body. These nerves convey motor, sensory, and autonomic information, enabling us to move, feel, and respond to changes in our environment. They form a complex network that extends from the CNS to muscles, glands, skin, and internal organs, allowing for coordinated responses and functions throughout the body. Damage or injury to peripheral nerves can result in various neurological symptoms, such as numbness, weakness, or pain, depending on the type and severity of the damage.

Demyelinating diseases are a group of disorders that are characterized by damage to the myelin sheath, which is the protective covering surrounding nerve fibers in the brain, optic nerves, and spinal cord. Myelin is essential for the rapid transmission of nerve impulses, and its damage results in disrupted communication between the brain and other parts of the body.

The most common demyelinating disease is multiple sclerosis (MS), where the immune system mistakenly attacks the myelin sheath. Other demyelinating diseases include:

1. Acute Disseminated Encephalomyelitis (ADEM): An autoimmune disorder that typically follows a viral infection or vaccination, causing widespread inflammation and demyelination in the brain and spinal cord.
2. Neuromyelitis Optica (NMO) or Devic's Disease: A rare autoimmune disorder that primarily affects the optic nerves and spinal cord, leading to severe vision loss and motor disability.
3. Transverse Myelitis: Inflammation of the spinal cord causing damage to both sides of one level (segment) of the spinal cord, resulting in various neurological symptoms such as muscle weakness, numbness, or pain, depending on which part of the spinal cord is affected.
4. Guillain-Barré Syndrome: An autoimmune disorder that causes rapid-onset muscle weakness, often beginning in the legs and spreading to the upper body, including the face and breathing muscles. It occurs when the immune system attacks the peripheral nerves' myelin sheath.
5. Central Pontine Myelinolysis (CPM): A rare neurological disorder caused by rapid shifts in sodium levels in the blood, leading to damage to the myelin sheath in a specific area of the brainstem called the pons.

These diseases can result in various symptoms, such as muscle weakness, numbness, vision loss, difficulty with balance and coordination, and cognitive impairment, depending on the location and extent of the demyelination. Treatment typically focuses on managing symptoms, modifying the immune system's response, and promoting nerve regeneration and remyelination when possible.

An axon is a long, slender extension of a neuron (a type of nerve cell) that conducts electrical impulses (nerve impulses) away from the cell body to target cells, such as other neurons or muscle cells. Axons can vary in length from a few micrometers to over a meter long and are typically surrounded by a myelin sheath, which helps to insulate and protect the axon and allows for faster transmission of nerve impulses.

Axons play a critical role in the functioning of the nervous system, as they provide the means by which neurons communicate with one another and with other cells in the body. Damage to axons can result in serious neurological problems, such as those seen in spinal cord injuries or neurodegenerative diseases like multiple sclerosis.

The sciatic nerve is the largest and longest nerve in the human body, running from the lower back through the buttocks and down the legs to the feet. It is formed by the union of the ventral rami (branches) of the L4 to S3 spinal nerves. The sciatic nerve provides motor and sensory innervation to various muscles and skin areas in the lower limbs, including the hamstrings, calf muscles, and the sole of the foot. Sciatic nerve disorders or injuries can result in symptoms such as pain, numbness, tingling, or weakness in the lower back, hips, legs, and feet, known as sciatica.

The Central Nervous System (CNS) is the part of the nervous system that consists of the brain and spinal cord. It is called the "central" system because it receives information from, and sends information to, the rest of the body through peripheral nerves, which make up the Peripheral Nervous System (PNS).

The CNS is responsible for processing sensory information, controlling motor functions, and regulating various autonomic processes like heart rate, respiration, and digestion. The brain, as the command center of the CNS, interprets sensory stimuli, formulates thoughts, and initiates actions. The spinal cord serves as a conduit for nerve impulses traveling to and from the brain and the rest of the body.

The CNS is protected by several structures, including the skull (which houses the brain) and the vertebral column (which surrounds and protects the spinal cord). Despite these protective measures, the CNS remains vulnerable to injury and disease, which can have severe consequences due to its crucial role in controlling essential bodily functions.

Electron microscopy (EM) is a type of microscopy that uses a beam of electrons to create an image of the sample being examined, resulting in much higher magnification and resolution than light microscopy. There are several types of electron microscopy, including transmission electron microscopy (TEM), scanning electron microscopy (SEM), and reflection electron microscopy (REM).

In TEM, a beam of electrons is transmitted through a thin slice of the sample, and the electrons that pass through the sample are focused to form an image. This technique can provide detailed information about the internal structure of cells, viruses, and other biological specimens, as well as the composition and structure of materials at the atomic level.

In SEM, a beam of electrons is scanned across the surface of the sample, and the electrons that are scattered back from the surface are detected to create an image. This technique can provide information about the topography and composition of surfaces, as well as the structure of materials at the microscopic level.

REM is a variation of SEM in which the beam of electrons is reflected off the surface of the sample, rather than scattered back from it. This technique can provide information about the surface chemistry and composition of materials.

Electron microscopy has a wide range of applications in biology, medicine, and materials science, including the study of cellular structure and function, disease diagnosis, and the development of new materials and technologies.

Myelin-Oligodendrocyte Glycoprotein (MOG) is a protein found exclusively on the outermost layer of myelin sheath in the central nervous system (CNS). The myelin sheath is a fatty substance that surrounds and insulates nerve fibers, allowing for efficient and rapid transmission of electrical signals. MOG plays a crucial role in maintaining the integrity and structure of the myelin sheath. It is involved in the adhesion of oligodendrocytes to the surface of neuronal axons and contributes to the stability of the compact myelin structure. Autoimmune reactions against MOG have been implicated in certain inflammatory demyelinating diseases, such as optic neuritis, transverse myelitis, and acute disseminated encephalomyelitis (ADEM).

A Compact Disc (CD) is not a medical term, but rather a term used in technology and electronics. It is a small, flat, circular piece of optical storage media that can hold digital data such as music, video, or computer files. The medical field does not use compact discs for storing patient records or other medical information, as there are more secure and efficient methods available for electronic health records (EHRs).

Autoimmune encephalomyelitis (EAE) is a model of inflammatory demyelinating disease used in medical research to study the mechanisms of multiple sclerosis (MS) and develop new therapies. It is experimentally induced in laboratory animals, typically mice or rats, through immunization with myelin antigens or T-cell transfer. The resulting immune response leads to inflammation, demyelination, and neurological dysfunction in the central nervous system (CNS), mimicking certain aspects of MS.

EAE is a valuable tool for understanding the pathogenesis of MS and testing potential treatments. However, it is essential to recognize that EAE is an experimental model and may not fully recapitulate all features of human autoimmune encephalomyelitis.

Myelinated nerve fibers are neuronal processes that are surrounded by a myelin sheath, a fatty insulating substance that is produced by Schwann cells in the peripheral nervous system and oligodendrocytes in the central nervous system. This myelin sheath helps to increase the speed of electrical impulse transmission, also known as action potentials, along the nerve fiber. The myelin sheath has gaps called nodes of Ranvier where the electrical impulses can jump from one node to the next, which also contributes to the rapid conduction of signals. Myelinated nerve fibers are typically found in the peripheral nerves and the optic nerve, but not in the central nervous system (CNS) tracts that are located within the brain and spinal cord.

Multiple Sclerosis (MS) is a chronic autoimmune disease that affects the central nervous system (CNS), which includes the brain, spinal cord, and optic nerves. In MS, the immune system mistakenly attacks the protective covering of nerve fibers, called myelin, leading to damage and scarring (sclerosis). This results in disrupted communication between the brain and the rest of the body, causing a variety of neurological symptoms that can vary widely from person to person.

The term "multiple" refers to the numerous areas of scarring that occur throughout the CNS in this condition. The progression, severity, and specific symptoms of MS are unpredictable and may include vision problems, muscle weakness, numbness or tingling, difficulty with balance and coordination, cognitive impairment, and mood changes. There is currently no cure for MS, but various treatments can help manage symptoms, modify the course of the disease, and improve quality of life for those affected.

2,3'-Cyclic Nucleotide 3'-Phosphodiesterase (CNP) is an enzyme that specifically hydrolyzes 2',3'-cyclic nucleotides to 2'-nucleotide monophosphates. It plays a crucial role in regulating the levels of intracellular second messengers, such as cyclic guanosine monophosphate (cGMP) and cyclic adenosine monophosphate (cAMP), which are involved in various cellular processes including signal transduction, gene expression, and metabolism.

CNP has two isoforms, CNP1 and CNP2, which differ in their tissue distribution and substrate specificity. CNP1 is predominantly expressed in the central nervous system (CNS) and preferentially hydrolyzes cGMP, while CNP2 is widely distributed and hydrolyzes both cGMP and cAMP with similar efficiency.

Mutations in the gene encoding CNP1 have been associated with certain neurological disorders, such as spastic paraplegia type 5 (SPG5), a hereditary condition characterized by progressive muscle weakness and stiffness in the lower limbs.

Molecular sequence data refers to the specific arrangement of molecules, most commonly nucleotides in DNA or RNA, or amino acids in proteins, that make up a biological macromolecule. This data is generated through laboratory techniques such as sequencing, and provides information about the exact order of the constituent molecules. This data is crucial in various fields of biology, including genetics, evolution, and molecular biology, allowing for comparisons between different organisms, identification of genetic variations, and studies of gene function and regulation.

Myelin P2 protein, also known as proteolipid protein 1 (PLP1), is a major structural component of the myelin sheath in the central nervous system. The myelin sheath is a protective and insulating layer that surrounds nerve cell fibers (axons), allowing for efficient and rapid transmission of electrical signals.

The P2 protein is a transmembrane protein, with four transmembrane domains, and it plays a crucial role in maintaining the stability and integrity of the myelin sheath. Mutations in the gene that encodes for this protein (PLP1) have been associated with several demyelinating diseases, including Pelizaeus-Merzbacher disease (PMD), a rare X-linked recessive disorder characterized by abnormalities in the development and maintenance of the myelin sheath.

The P2 protein is also involved in various cellular processes, such as signal transduction, ion transport, and immune response regulation. However, the precise mechanisms through which these functions are carried out remain to be fully elucidated.

The optic nerve, also known as the second cranial nerve, is the nerve that transmits visual information from the retina to the brain. It is composed of approximately one million nerve fibers that carry signals related to vision, such as light intensity and color, from the eye's photoreceptor cells (rods and cones) to the visual cortex in the brain. The optic nerve is responsible for carrying this visual information so that it can be processed and interpreted by the brain, allowing us to see and perceive our surroundings. Damage to the optic nerve can result in vision loss or impairment.

The spinal cord is a major part of the nervous system, extending from the brainstem and continuing down to the lower back. It is a slender, tubular bundle of nerve fibers (axons) and support cells (glial cells) that carries signals between the brain and the rest of the body. The spinal cord primarily serves as a conduit for motor information, which travels from the brain to the muscles, and sensory information, which travels from the body to the brain. It also contains neurons that can independently process and respond to information within the spinal cord without direct input from the brain.

The spinal cord is protected by the bony vertebral column (spine) and is divided into 31 segments: 8 cervical, 12 thoracic, 5 lumbar, 5 sacral, and 1 coccygeal. Each segment corresponds to a specific region of the body and gives rise to pairs of spinal nerves that exit through the intervertebral foramina at each level.

The spinal cord is responsible for several vital functions, including:

1. Reflexes: Simple reflex actions, such as the withdrawal reflex when touching a hot surface, are mediated by the spinal cord without involving the brain.
2. Muscle control: The spinal cord carries motor signals from the brain to the muscles, enabling voluntary movement and muscle tone regulation.
3. Sensory perception: The spinal cord transmits sensory information, such as touch, temperature, pain, and vibration, from the body to the brain for processing and awareness.
4. Autonomic functions: The sympathetic and parasympathetic divisions of the autonomic nervous system originate in the thoracolumbar and sacral regions of the spinal cord, respectively, controlling involuntary physiological responses like heart rate, blood pressure, digestion, and respiration.

Damage to the spinal cord can result in various degrees of paralysis or loss of sensation below the level of injury, depending on the severity and location of the damage.

Sulfoglycosphingolipids are a type of glycosphingolipid that contain a sulfate ester group in their carbohydrate moiety. They are important components of animal cell membranes and play a role in various biological processes, including cell recognition, signal transduction, and cell adhesion.

The most well-known sulfoglycosphingolipids are the sulfatides, which contain a 3'-sulfate ester on the galactose residue of the glycosphingolipid GalCer (galactosylceramide). Sulfatides are abundant in the nervous system and have been implicated in various neurological disorders.

Other sulfoglycosphingolipids include the seminolipids, which contain a 3'-sulfate ester on the galactose residue of lactosylceramide (Galβ1-4Glcβ1-Cer), and are found in high concentrations in the testis.

Abnormalities in sulfoglycosphingolipid metabolism have been associated with several genetic disorders, such as metachromatic leukodystrophy (MLD) and globoid cell leukodystrophy (GLD), which are characterized by progressive neurological deterioration.

An amino acid sequence is the specific order of amino acids in a protein or peptide molecule, formed by the linking of the amino group (-NH2) of one amino acid to the carboxyl group (-COOH) of another amino acid through a peptide bond. The sequence is determined by the genetic code and is unique to each type of protein or peptide. It plays a crucial role in determining the three-dimensional structure and function of proteins.

Molecular models are three-dimensional representations of molecular structures that are used in the field of molecular biology and chemistry to visualize and understand the spatial arrangement of atoms and bonds within a molecule. These models can be physical or computer-generated and allow researchers to study the shape, size, and behavior of molecules, which is crucial for understanding their function and interactions with other molecules.

Physical molecular models are often made up of balls (representing atoms) connected by rods or sticks (representing bonds). These models can be constructed manually using materials such as plastic or wooden balls and rods, or they can be created using 3D printing technology.

Computer-generated molecular models, on the other hand, are created using specialized software that allows researchers to visualize and manipulate molecular structures in three dimensions. These models can be used to simulate molecular interactions, predict molecular behavior, and design new drugs or chemicals with specific properties. Overall, molecular models play a critical role in advancing our understanding of molecular structures and their functions.

Protein conformation refers to the specific three-dimensional shape that a protein molecule assumes due to the spatial arrangement of its constituent amino acid residues and their associated chemical groups. This complex structure is determined by several factors, including covalent bonds (disulfide bridges), hydrogen bonds, van der Waals forces, and ionic bonds, which help stabilize the protein's unique conformation.

Protein conformations can be broadly classified into two categories: primary, secondary, tertiary, and quaternary structures. The primary structure represents the linear sequence of amino acids in a polypeptide chain. The secondary structure arises from local interactions between adjacent amino acid residues, leading to the formation of recurring motifs such as α-helices and β-sheets. Tertiary structure refers to the overall three-dimensional folding pattern of a single polypeptide chain, while quaternary structure describes the spatial arrangement of multiple folded polypeptide chains (subunits) that interact to form a functional protein complex.

Understanding protein conformation is crucial for elucidating protein function, as the specific three-dimensional shape of a protein directly influences its ability to interact with other molecules, such as ligands, nucleic acids, or other proteins. Any alterations in protein conformation due to genetic mutations, environmental factors, or chemical modifications can lead to loss of function, misfolding, aggregation, and disease states like neurodegenerative disorders and cancer.

Wallerian degeneration is a process that occurs following damage to the axons of neurons (nerve cells). After an axon is severed or traumatically injured, it undergoes a series of changes including fragmentation and removal of the distal segment of the axon, which is the part that is separated from the cell body. This process is named after Augustus Waller, who first described it in 1850.

The degenerative changes in the distal axon are characterized by the breakdown of the axonal cytoskeleton, the loss of myelin sheath (the fatty insulating material that surrounds and protects the axon), and the infiltration of macrophages to clear away the debris. These events lead to the degeneration of the distal axon segment, which is necessary for successful regeneration of the injured nerve.

Wallerian degeneration is a crucial process in the nervous system's response to injury, as it enables the regrowth of axons and the reestablishment of connections between neurons. However, if the regenerative capacity of the neuron is insufficient or the environment is not conducive to growth, functional recovery may be impaired, leading to long-term neurological deficits.

2,3'-Cyclic-nucleotide phosphodiesterases (PDEs) are a subclass of enzymes that belong to the family of phosphodiesterases. These enzymes are responsible for the hydrolysis of 2,3'-cyclic nucleotides, which are cyclic forms of nucleotides that act as second messengers in various cellular signaling pathways.

The two primary types of 2,3'-cyclic nucleotides are 2',3'-cGMP and 2',3'-cAMP, which are produced by the action of certain enzymes on their respective precursors, guanosine triphosphate (GTP) and adenosine triphosphate (ATP). These cyclic nucleotides play important roles in regulating various cellular processes, including metabolism, gene expression, and ion channel activity.

2,3'-Cyclic-nucleotide phosphodiesterases catalyze the hydrolysis of these cyclic nucleotides to their corresponding 5'-monophosphates, thereby terminating their signaling activity. There are several isoforms of 2,3'-cyclic-nucleotide PDEs that have been identified, each with distinct substrate specificities and regulatory properties.

Dysregulation of 2,3'-cyclic-nucleotide PDE activity has been implicated in various diseases, including cancer, cardiovascular disease, and neurological disorders. Therefore, these enzymes have emerged as important targets for the development of therapeutic agents that can modulate their activity and restore normal cellular function.

The brain is the central organ of the nervous system, responsible for receiving and processing sensory information, regulating vital functions, and controlling behavior, movement, and cognition. It is divided into several distinct regions, each with specific functions:

1. Cerebrum: The largest part of the brain, responsible for higher cognitive functions such as thinking, learning, memory, language, and perception. It is divided into two hemispheres, each controlling the opposite side of the body.
2. Cerebellum: Located at the back of the brain, it is responsible for coordinating muscle movements, maintaining balance, and fine-tuning motor skills.
3. Brainstem: Connects the cerebrum and cerebellum to the spinal cord, controlling vital functions such as breathing, heart rate, and blood pressure. It also serves as a relay center for sensory information and motor commands between the brain and the rest of the body.
4. Diencephalon: A region that includes the thalamus (a major sensory relay station) and hypothalamus (regulates hormones, temperature, hunger, thirst, and sleep).
5. Limbic system: A group of structures involved in emotional processing, memory formation, and motivation, including the hippocampus, amygdala, and cingulate gyrus.

The brain is composed of billions of interconnected neurons that communicate through electrical and chemical signals. It is protected by the skull and surrounded by three layers of membranes called meninges, as well as cerebrospinal fluid that provides cushioning and nutrients.

I'm sorry for any confusion, but "Jimpy" is not a recognized medical term or condition associated with mice in the field of veterinary medicine. It may be a colloquial or informal term used to describe a particular characteristic or behavior in mice, but without further context, it's challenging to provide an accurate definition. If you could provide more information about where you encountered this term or its intended meaning, I would be happy to help you further.

Protein folding is the process by which a protein molecule naturally folds into its three-dimensional structure, following the synthesis of its amino acid chain. This complex process is determined by the sequence and properties of the amino acids, as well as various environmental factors such as temperature, pH, and the presence of molecular chaperones. The final folded conformation of a protein is crucial for its proper function, as it enables the formation of specific interactions between different parts of the molecule, which in turn define its biological activity. Protein misfolding can lead to various diseases, including neurodegenerative disorders such as Alzheimer's and Parkinson's disease.

Charcot-Marie-Tooth disease (CMT) is a group of inherited disorders that cause nerve damage, primarily affecting the peripheral nerves. These are the nerves that transmit signals between the brain and spinal cord to the rest of the body. CMT affects both motor and sensory nerves, leading to muscle weakness and atrophy, as well as numbness or tingling in the hands and feet.

The disease is named after the three physicians who first described it: Jean-Martin Charcot, Pierre Marie, and Howard Henry Tooth. CMT is characterized by its progressive nature, meaning symptoms typically worsen over time, although the rate of progression can vary significantly among individuals.

There are several types of CMT, classified based on their genetic causes and patterns of inheritance. The two most common forms are CMT1 and CMT2:

1. CMT1: This form is caused by mutations in the genes responsible for the myelin sheath, which insulates peripheral nerves and allows for efficient signal transmission. As a result, demyelination occurs, slowing down nerve impulses and causing muscle weakness, particularly in the lower limbs. Symptoms usually begin in childhood or adolescence and include foot drop, high arches, and hammertoes.
2. CMT2: This form is caused by mutations in the genes responsible for the axons, the nerve fibers that transmit signals within peripheral nerves. As a result, axonal degeneration occurs, leading to muscle weakness and atrophy. Symptoms usually begin in early adulthood and progress more slowly than CMT1. They primarily affect the lower limbs but can also involve the hands and arms.

Diagnosis of CMT typically involves a combination of clinical evaluation, family history, nerve conduction studies, and genetic testing. While there is no cure for CMT, treatment focuses on managing symptoms and maintaining mobility and function through physical therapy, bracing, orthopedic surgery, and pain management.

Cerebrosides are a type of sphingolipid, which are lipids that contain sphingosine. They are major components of the outer layer of cell membranes and are particularly abundant in the nervous system. Cerebrosides are composed of a ceramide molecule (a fatty acid attached to sphingosine) and a sugar molecule, usually either glucose or galactose.

Glycosphingolipids that contain a ceramide with a single sugar residue are called cerebrosides. Those that contain more complex oligosaccharide chains are called gangliosides. Cerebrosides play important roles in cell recognition, signal transduction, and cell adhesion.

Abnormalities in the metabolism of cerebrosides can lead to various genetic disorders, such as Gaucher's disease, Krabbe disease, and Fabry disease. These conditions are characterized by the accumulation of cerebrosides or their breakdown products in various tissues, leading to progressive damage and dysfunction.

Cuprizone is not a medical condition or disease, but rather a chemical compound that is used in laboratory settings for research purposes. Cuprizone, also known as bis-cyclohexanone oxaldihydrazone, is a copper chelator, which means it can bind to and remove copper ions from various substances.

In research, cuprizone is often used to induce demyelination in animal models of multiple sclerosis (MS) and other neurological disorders. Demyelination refers to the loss or damage of the myelin sheath, which is a fatty substance that surrounds and protects nerve fibers in the brain and spinal cord. When cuprizone is added to the diet of laboratory animals such as mice, it can cause demyelination in specific areas of the brain, making it a useful tool for studying the mechanisms underlying MS and other demyelinating diseases.

It's important to note that while cuprizone is a valuable research tool, it is not used as a medical treatment or therapy for any human conditions.

Nerve regeneration is the process of regrowth and restoration of functional nerve connections following damage or injury to the nervous system. This complex process involves various cellular and molecular events, such as the activation of support cells called glia, the sprouting of surviving nerve fibers (axons), and the reformation of neural circuits. The goal of nerve regeneration is to enable the restoration of normal sensory, motor, and autonomic functions impaired due to nerve damage or injury.

Ganglioside Galactosyltransferase is a type of enzyme that plays a role in the biosynthesis of gangliosides, which are complex glycosphingolipids found in high concentrations in the outer leaflet of the plasma membrane of cells, particularly in the nervous system.

Gangliosides contain one or more sialic acid residues and are involved in various cellular processes such as cell recognition, signal transduction, and cell adhesion. The enzyme Ganglioside Galactosyltransferase catalyzes the transfer of a galactose molecule from a donor (usually UDP-galactose) to an acceptor molecule, which is a specific ganglioside substrate.

The reaction facilitated by Ganglioside Galactosyltransferase results in the formation of a new glycosidic bond and the production of more complex gangliosides. Defects in this enzyme have been associated with certain neurological disorders, highlighting its importance in maintaining normal brain function.

A peptide fragment is a short chain of amino acids that is derived from a larger peptide or protein through various biological or chemical processes. These fragments can result from the natural breakdown of proteins in the body during regular physiological processes, such as digestion, or they can be produced experimentally in a laboratory setting for research or therapeutic purposes.

Peptide fragments are often used in research to map the structure and function of larger peptides and proteins, as well as to study their interactions with other molecules. In some cases, peptide fragments may also have biological activity of their own and can be developed into drugs or diagnostic tools. For example, certain peptide fragments derived from hormones or neurotransmitters may bind to receptors in the body and mimic or block the effects of the full-length molecule.

Secondary protein structure refers to the local spatial arrangement of amino acid chains in a protein, typically described as regular repeating patterns held together by hydrogen bonds. The two most common types of secondary structures are the alpha-helix (α-helix) and the beta-pleated sheet (β-sheet). In an α-helix, the polypeptide chain twists around itself in a helical shape, with each backbone atom forming a hydrogen bond with the fourth amino acid residue along the chain. This forms a rigid rod-like structure that is resistant to bending or twisting forces. In β-sheets, adjacent segments of the polypeptide chain run parallel or antiparallel to each other and are connected by hydrogen bonds, forming a pleated sheet-like arrangement. These secondary structures provide the foundation for the formation of tertiary and quaternary protein structures, which determine the overall three-dimensional shape and function of the protein.

Tertiary protein structure refers to the three-dimensional arrangement of all the elements (polypeptide chains) of a single protein molecule. It is the highest level of structural organization and results from interactions between various side chains (R groups) of the amino acids that make up the protein. These interactions, which include hydrogen bonds, ionic bonds, van der Waals forces, and disulfide bridges, give the protein its unique shape and stability, which in turn determines its function. The tertiary structure of a protein can be stabilized by various factors such as temperature, pH, and the presence of certain ions. Any changes in these factors can lead to denaturation, where the protein loses its tertiary structure and thus its function.

Neuroglia, also known as glial cells or simply glia, are non-neuronal cells that provide support and protection for neurons in the nervous system. They maintain homeostasis, form myelin sheaths around nerve fibers, and provide structural support. They also play a role in the immune response of the central nervous system. Some types of neuroglia include astrocytes, oligodendrocytes, microglia, and ependymal cells.

In the context of medical terminology, "lenses" generally refers to optical lenses used in various medical devices and instruments. These lenses are typically made of glass or plastic and are designed to refract (bend) light in specific ways to help magnify, focus, or redirect images. Here are some examples:

1. In ophthalmology and optometry, lenses are used in eyeglasses, contact lenses, and ophthalmic instruments to correct vision problems like myopia (nearsightedness), hypermetropia (farsightedness), astigmatism, or presbyopia.
2. In surgical microscopes, lenses are used to provide a magnified and clear view of the operating field during microsurgical procedures like ophthalmic, neurosurgical, or ENT (Ear, Nose, Throat) surgeries.
3. In endoscopes and laparoscopes, lenses are used to transmit light and images from inside the body during minimally invasive surgical procedures.
4. In ophthalmic diagnostic instruments like slit lamps, lenses are used to examine various structures of the eye in detail.

In summary, "lenses" in medical terminology refer to optical components that help manipulate light to aid in diagnosis, treatment, or visual correction.

Brain chemistry refers to the chemical processes that occur within the brain, particularly those involving neurotransmitters, neuromodulators, and neuropeptides. These chemicals are responsible for transmitting signals between neurons (nerve cells) in the brain, allowing for various cognitive, emotional, and physical functions.

Neurotransmitters are chemical messengers that transmit signals across the synapse (the tiny gap between two neurons). Examples of neurotransmitters include dopamine, serotonin, norepinephrine, GABA (gamma-aminobutyric acid), and glutamate. Each neurotransmitter has a specific role in brain function, such as regulating mood, motivation, attention, memory, and movement.

Neuromodulators are chemicals that modify the effects of neurotransmitters on neurons. They can enhance or inhibit the transmission of signals between neurons, thereby modulating brain activity. Examples of neuromodulators include acetylcholine, histamine, and substance P.

Neuropeptides are small protein-like molecules that act as neurotransmitters or neuromodulators. They play a role in various physiological functions, such as pain perception, stress response, and reward processing. Examples of neuropeptides include endorphins, enkephalins, and oxytocin.

Abnormalities in brain chemistry can lead to various neurological and psychiatric conditions, such as depression, anxiety disorders, schizophrenia, Parkinson's disease, and Alzheimer's disease. Understanding brain chemistry is crucial for developing effective treatments for these conditions.

Circular dichroism (CD) is a technique used in physics and chemistry to study the structure of molecules, particularly large biological molecules such as proteins and nucleic acids. It measures the difference in absorption of left-handed and right-handed circularly polarized light by a sample. This difference in absorption can provide information about the three-dimensional structure of the molecule, including its chirality or "handedness."

In more technical terms, CD is a form of spectroscopy that measures the differential absorption of left and right circularly polarized light as a function of wavelength. The CD signal is measured in units of millidegrees (mdeg) and can be positive or negative, depending on the type of chromophore and its orientation within the molecule.

CD spectra can provide valuable information about the secondary and tertiary structure of proteins, as well as the conformation of nucleic acids. For example, alpha-helical proteins typically exhibit a strong positive band near 190 nm and two negative bands at around 208 nm and 222 nm, while beta-sheet proteins show a strong positive band near 195 nm and two negative bands at around 217 nm and 175 nm.

CD spectroscopy is a powerful tool for studying the structural changes that occur in biological molecules under different conditions, such as temperature, pH, or the presence of ligands or other molecules. It can also be used to monitor the folding and unfolding of proteins, as well as the binding of drugs or other small molecules to their targets.

Transmission electron microscopy (TEM) is a type of microscopy in which an electron beam is transmitted through a ultra-thin specimen, interacting with it as it passes through. An image is formed from the interaction of the electrons with the specimen; the image is then magnified and visualized on a fluorescent screen or recorded on an electronic detector (or photographic film in older models).

TEM can provide high-resolution, high-magnification images that can reveal the internal structure of specimens including cells, viruses, and even molecules. It is widely used in biological and materials science research to investigate the ultrastructure of cells, tissues and materials. In medicine, TEM is used for diagnostic purposes in fields such as virology and bacteriology.

It's important to note that preparing a sample for TEM is a complex process, requiring specialized techniques to create thin (50-100 nm) specimens. These include cutting ultrathin sections of embedded samples using an ultramicrotome, staining with heavy metal salts, and positive staining or negative staining methods.

Protein binding, in the context of medical and biological sciences, refers to the interaction between a protein and another molecule (known as the ligand) that results in a stable complex. This process is often reversible and can be influenced by various factors such as pH, temperature, and concentration of the involved molecules.

In clinical chemistry, protein binding is particularly important when it comes to drugs, as many of them bind to proteins (especially albumin) in the bloodstream. The degree of protein binding can affect a drug's distribution, metabolism, and excretion, which in turn influence its therapeutic effectiveness and potential side effects.

Protein-bound drugs may be less available for interaction with their target tissues, as only the unbound or "free" fraction of the drug is active. Therefore, understanding protein binding can help optimize dosing regimens and minimize adverse reactions.

X-ray diffraction (XRD) is not strictly a medical definition, but it is a technique commonly used in the field of medical research and diagnostics. XRD is a form of analytical spectroscopy that uses the phenomenon of X-ray diffraction to investigate the crystallographic structure of materials. When a beam of X-rays strikes a crystal, it is scattered in specific directions and with specific intensities that are determined by the arrangement of atoms within the crystal. By measuring these diffraction patterns, researchers can determine the crystal structures of various materials, including biological macromolecules such as proteins and viruses.

In the medical field, XRD is often used to study the structure of drugs and drug candidates, as well as to analyze the composition and structure of tissues and other biological samples. For example, XRD can be used to investigate the crystal structures of calcium phosphate minerals in bone tissue, which can provide insights into the mechanisms of bone formation and disease. Additionally, XRD is sometimes used in the development of new medical imaging techniques, such as phase-contrast X-ray imaging, which has the potential to improve the resolution and contrast of traditional X-ray images.

Nerve tissue proteins are specialized proteins found in the nervous system that provide structural and functional support to nerve cells, also known as neurons. These proteins include:

1. Neurofilaments: These are type IV intermediate filaments that provide structural support to neurons and help maintain their shape and size. They are composed of three subunits - NFL (light), NFM (medium), and NFH (heavy).

2. Neuronal Cytoskeletal Proteins: These include tubulins, actins, and spectrins that provide structural support to the neuronal cytoskeleton and help maintain its integrity.

3. Neurotransmitter Receptors: These are specialized proteins located on the postsynaptic membrane of neurons that bind neurotransmitters released by presynaptic neurons, triggering a response in the target cell.

4. Ion Channels: These are transmembrane proteins that regulate the flow of ions across the neuronal membrane and play a crucial role in generating and transmitting electrical signals in neurons.

5. Signaling Proteins: These include enzymes, receptors, and adaptor proteins that mediate intracellular signaling pathways involved in neuronal development, differentiation, survival, and death.

6. Adhesion Proteins: These are cell surface proteins that mediate cell-cell and cell-matrix interactions, playing a crucial role in the formation and maintenance of neural circuits.

7. Extracellular Matrix Proteins: These include proteoglycans, laminins, and collagens that provide structural support to nerve tissue and regulate neuronal migration, differentiation, and survival.

'Poisonous fishes' are species of fish that contain toxic substances in their bodies, which can cause harm or injury to other organisms, including humans. These toxins can be present in various parts of the fish, such as the flesh, skin, organs, or even in the form of venomous spines.

There are several types of poisonous fishes, including:

1. Pufferfish (Fugu): These fish contain a potent neurotoxin called tetrodotoxin (TTX) in their organs, especially the liver and ovaries. TTX is highly toxic and can cause paralysis and death if ingested in even small amounts.
2. Stonefish: Stonefishes are venomous fishes that have sharp, spiny dorsal fins that can inject a painful toxin into the skin when stepped on or touched. The venom can cause severe pain, swelling, and tissue damage, and in some cases, it can lead to respiratory failure and death.
3. Blue-ringed octopuses: While not technically fish, blue-ringed octopuses are often included in discussions of poisonous marine life. They have venom glands that produce a powerful neurotoxin called tetrodotoxin (TTX), which can cause paralysis and death if it enters the bloodstream.
4. Cone snails: Cone snails are predatory mollusks that use a harpoon-like tooth to inject venom into their prey. Some species of cone snail have venom that contains powerful neurotoxins, which can cause paralysis and death in humans.
5. Lionfish: Lionfish are venomous fishes that have spines on their dorsal, pelvic, and anal fins that can inject a painful toxin into the skin when touched or stepped on. The venom can cause pain, swelling, and other symptoms, but it is rarely fatal to humans.

It's important to note that many species of fish can become toxic if they consume harmful algae blooms (HABs) or other contaminants in their environment. These "toxic fishes" are not considered poisonous by definition, as their toxicity is not inherent to their biology.

Small angle scattering (SAS) in the context of medical physics refers to a technique used to study the structure of non-crystalline materials at the nanoscale. It is called "small angle" because the scattering angles are very small, typically less than a few degrees. This occurs when X-rays, neutrons, or electrons interact with a sample and are scattered in various directions. The intensity of the scattered radiation is measured as a function of the scattering angle, which provides information about the size, shape, and spatial distribution of the nanostructures within the sample. SAS can be used to study a wide range of biological and materials science samples, including proteins, polymers, colloids, and porous materials.

"Cells, cultured" is a medical term that refers to cells that have been removed from an organism and grown in controlled laboratory conditions outside of the body. This process is called cell culture and it allows scientists to study cells in a more controlled and accessible environment than they would have inside the body. Cultured cells can be derived from a variety of sources, including tissues, organs, or fluids from humans, animals, or cell lines that have been previously established in the laboratory.

Cell culture involves several steps, including isolation of the cells from the tissue, purification and characterization of the cells, and maintenance of the cells in appropriate growth conditions. The cells are typically grown in specialized media that contain nutrients, growth factors, and other components necessary for their survival and proliferation. Cultured cells can be used for a variety of purposes, including basic research, drug development and testing, and production of biological products such as vaccines and gene therapies.

It is important to note that cultured cells may behave differently than they do in the body, and results obtained from cell culture studies may not always translate directly to human physiology or disease. Therefore, it is essential to validate findings from cell culture experiments using additional models and ultimately in clinical trials involving human subjects.

I'm happy to help! "Rats, Inbred Lew" is a specific strain of laboratory rats that have been inbred for research purposes. The "Lew" part of the name refers to the location where they were first developed, the Lewis Institute in Lake Bluff, Illinois, USA.

Inbreeding is a process of mating closely related individuals over many generations to create a genetically homogeneous population. This results in a high degree of genetic similarity among members of the strain, making them ideal for use as experimental models because any differences observed between individuals are more likely to be due to the experimental manipulation rather than genetic variation.

Inbred Lew rats have been widely used in biomedical research, particularly in studies related to hypertension and cardiovascular disease. They exhibit a number of unique characteristics that make them useful for these types of studies, including their susceptibility to developing high blood pressure when fed a high-salt diet or given certain drugs.

It's important to note that while inbred strains like Lew rats can be very useful tools for researchers, they are not perfect models for human disease. Because they have been bred in a controlled environment and selected for specific traits, they may not respond to experimental manipulations in the same way that humans or other animals would. Therefore, it's important to interpret findings from these studies with caution and consider multiple lines of evidence before drawing any firm conclusions.

Early Growth Response Protein 2 (EGR2) is a transcription factor that belongs to the EGR family of proteins, which are involved in various biological processes such as cell proliferation, differentiation, and apoptosis. EGR2 is specifically known to play crucial roles in the development and function of the nervous system, including the regulation of neuronal survival, axon guidance, and myelination. It is also expressed in immune cells and has been implicated in the regulation of immune responses. Mutations in the EGR2 gene have been associated with certain neurological disorders and diseases, such as Charcot-Marie-Tooth disease type 1B and congenital hypomyelinating neuropathy.

Demyelinating autoimmune diseases of the central nervous system (CNS) are a group of disorders characterized by inflammation and damage to the myelin sheath, which is the protective covering that surrounds nerve fibers in the brain and spinal cord. This damage can result in various neurological symptoms, including muscle weakness, sensory loss, vision problems, and cognitive impairment.

The most common demyelinating autoimmune disease of the CNS is multiple sclerosis (MS), which affects approximately 2.3 million people worldwide. Other examples include neuromyelitis optica spectrum disorder (NMOSD), acute disseminated encephalomyelitis (ADEM), and transverse myelitis.

These conditions are thought to arise when the immune system mistakenly attacks the myelin sheath, leading to inflammation, damage, and scarring (sclerosis) in the CNS. The exact cause of this autoimmune response is not fully understood, but it is believed to involve a complex interplay between genetic, environmental, and immunological factors.

Treatment for demyelinating autoimmune diseases of the CNS typically involves a combination of medications to manage symptoms, reduce inflammation, and modify the course of the disease. These may include corticosteroids, immunosuppressive drugs, and disease-modifying therapies (DMTs) that target specific components of the immune system.

C57BL/6 (C57 Black 6) is an inbred strain of laboratory mouse that is widely used in biomedical research. The term "inbred" refers to a strain of animals where matings have been carried out between siblings or other closely related individuals for many generations, resulting in a population that is highly homozygous at most genetic loci.

The C57BL/6 strain was established in 1920 by crossing a female mouse from the dilute brown (DBA) strain with a male mouse from the black strain. The resulting offspring were then interbred for many generations to create the inbred C57BL/6 strain.

C57BL/6 mice are known for their robust health, longevity, and ease of handling, making them a popular choice for researchers. They have been used in a wide range of biomedical research areas, including studies of cancer, immunology, neuroscience, cardiovascular disease, and metabolism.

One of the most notable features of the C57BL/6 strain is its sensitivity to certain genetic modifications, such as the introduction of mutations that lead to obesity or impaired glucose tolerance. This has made it a valuable tool for studying the genetic basis of complex diseases and traits.

Overall, the C57BL/6 inbred mouse strain is an important model organism in biomedical research, providing a valuable resource for understanding the genetic and molecular mechanisms underlying human health and disease.

A base sequence in the context of molecular biology refers to the specific order of nucleotides in a DNA or RNA molecule. In DNA, these nucleotides are adenine (A), guanine (G), cytosine (C), and thymine (T). In RNA, uracil (U) takes the place of thymine. The base sequence contains genetic information that is transcribed into RNA and ultimately translated into proteins. It is the exact order of these bases that determines the genetic code and thus the function of the DNA or RNA molecule.

Protein denaturation is a process in which the native structure of a protein is altered, leading to loss of its biological activity. This can be caused by various factors such as changes in temperature, pH, or exposure to chemicals or radiation. The three-dimensional shape of a protein is crucial for its function, and denaturation causes the protein to lose this shape, resulting in impaired or complete loss of function. Denaturation is often irreversible and can lead to the aggregation of proteins, which can have negative effects on cellular function and can contribute to diseases such as Alzheimer's and Parkinson's.

The Peripheral Nervous System (PNS) is that part of the nervous system which lies outside of the brain and spinal cord. It includes all the nerves and ganglia ( clusters of neurons) outside of the central nervous system (CNS). The PNS is divided into two components: the somatic nervous system and the autonomic nervous system.

The somatic nervous system is responsible for transmitting sensory information from the skin, muscles, and joints to the CNS, and for controlling voluntary movements of the skeletal muscles.

The autonomic nervous system, on the other hand, controls involuntary actions, such as heart rate, digestion, respiratory rate, salivation, perspiration, pupillary dilation, and sexual arousal. It is further divided into the sympathetic and parasympathetic systems, which generally have opposing effects and maintain homeostasis in the body.

Damage to the peripheral nervous system can result in various medical conditions such as neuropathies, neuritis, plexopathies, and radiculopathies, leading to symptoms like numbness, tingling, pain, weakness, or loss of reflexes in the affected area.

Transgenic mice are genetically modified rodents that have incorporated foreign DNA (exogenous DNA) into their own genome. This is typically done through the use of recombinant DNA technology, where a specific gene or genetic sequence of interest is isolated and then introduced into the mouse embryo. The resulting transgenic mice can then express the protein encoded by the foreign gene, allowing researchers to study its function in a living organism.

The process of creating transgenic mice usually involves microinjecting the exogenous DNA into the pronucleus of a fertilized egg, which is then implanted into a surrogate mother. The offspring that result from this procedure are screened for the presence of the foreign DNA, and those that carry the desired genetic modification are used to establish a transgenic mouse line.

Transgenic mice have been widely used in biomedical research to model human diseases, study gene function, and test new therapies. They provide a valuable tool for understanding complex biological processes and developing new treatments for a variety of medical conditions.

"Cattle" is a term used in the agricultural and veterinary fields to refer to domesticated animals of the genus *Bos*, primarily *Bos taurus* (European cattle) and *Bos indicus* (Zebu). These animals are often raised for meat, milk, leather, and labor. They are also known as bovines or cows (for females), bulls (intact males), and steers/bullocks (castrated males). However, in a strict medical definition, "cattle" does not apply to humans or other animals.

Hereditary Sensory and Motor Neuropathy (HSMN) is a group of inherited disorders that affect the peripheral nerves, which are the nerves outside the brain and spinal cord. These nerves transmit information between the brain and muscles, as well as sensations such as touch, pain, heat, and cold.

HSMN is characterized by progressive degeneration of these peripheral nerves, leading to muscle weakness, numbness, and tingling sensations, particularly in the hands and feet. The condition can also affect the autonomic nervous system, which controls involuntary functions such as heart rate, blood pressure, and digestion.

HSMN is caused by genetic mutations that are inherited from one or both parents. There are several types of HSMN, each with its own specific symptoms, severity, and pattern of inheritance. The most common form is Charcot-Marie-Tooth disease (CMT), which affects both motor and sensory nerves.

Treatment for HSMN typically focuses on managing the symptoms and preventing complications. This may include physical therapy, bracing or orthopedic surgery to support weakened muscles, pain management, and lifestyle modifications such as avoiding activities that aggravate symptoms. There is currently no cure for HSMN, but ongoing research is aimed at developing new treatments and therapies to slow or halt the progression of the disease.

"Miniaturization" is not a term that has a specific medical definition. However, in a broader context, it refers to the process of creating smaller versions of something, usually with the aim of improving functionality, efficiency, or ease of use. In medicine, this concept can be applied to various fields such as medical devices, surgical techniques, and diagnostic tools.

For instance, in interventional radiology, miniaturization refers to the development of smaller and less invasive catheters, wires, and other devices used during minimally invasive procedures. This allows for improved patient outcomes, reduced recovery time, and lower risks of complications compared to traditional open surgical procedures.

Similarly, in pathology, miniaturization can refer to the use of smaller tissue samples or biopsies for diagnostic testing, which can reduce the need for more invasive procedures while still providing accurate results.

Overall, while "miniaturization" is not a medical term per se, it reflects an ongoing trend in medicine towards developing more efficient and less invasive technologies and techniques to improve patient care.

Equipment design, in the medical context, refers to the process of creating and developing medical equipment and devices, such as surgical instruments, diagnostic machines, or assistive technologies. This process involves several stages, including:

1. Identifying user needs and requirements
2. Concept development and brainstorming
3. Prototyping and testing
4. Design for manufacturing and assembly
5. Safety and regulatory compliance
6. Verification and validation
7. Training and support

The goal of equipment design is to create safe, effective, and efficient medical devices that meet the needs of healthcare providers and patients while complying with relevant regulations and standards. The design process typically involves a multidisciplinary team of engineers, clinicians, designers, and researchers who work together to develop innovative solutions that improve patient care and outcomes.

A "knockout" mouse is a genetically engineered mouse in which one or more genes have been deleted or "knocked out" using molecular biology techniques. This allows researchers to study the function of specific genes and their role in various biological processes, as well as potential associations with human diseases. The mice are generated by introducing targeted DNA modifications into embryonic stem cells, which are then used to create a live animal. Knockout mice have been widely used in biomedical research to investigate gene function, disease mechanisms, and potential therapeutic targets.

I'm sorry for any confusion, but "holography" is not a term that has a medical definition. Holography is a technique used to create 3D images, typically using laser light. It is commonly used in non-medical fields such as data storage, art, and security features on credit cards and banknotes. I'm here to help with medical information, so please feel free to ask me about any medical topics or questions you might have!

X-ray crystallography is a technique used in structural biology to determine the three-dimensional arrangement of atoms in a crystal lattice. In this method, a beam of X-rays is directed at a crystal and diffracts, or spreads out, into a pattern of spots called reflections. The intensity and angle of each reflection are measured and used to create an electron density map, which reveals the position and type of atoms in the crystal. This information can be used to determine the molecular structure of a compound, including its shape, size, and chemical bonds. X-ray crystallography is a powerful tool for understanding the structure and function of biological macromolecules such as proteins and nucleic acids.

Proteolipids are a type of complex lipid-containing proteins that are insoluble in water and have a high content of hydrophobic amino acids. They are primarily found in the plasma membrane of cells, where they play important roles in maintaining the structural integrity and function of the membrane. Proteolipids are also found in various organelles, including mitochondria, lysosomes, and peroxisomes.

Proteolipids are composed of a hydrophobic protein core that is tightly associated with a lipid bilayer through non-covalent interactions. The protein component of proteolipids typically contains several transmembrane domains that span the lipid bilayer, as well as hydrophilic regions that face the cytoplasm or the lumen of organelles.

Proteolipids have been implicated in various cellular processes, including signal transduction, membrane trafficking, and ion transport. They are also associated with several neurological disorders, such as Alzheimer's disease, Parkinson's disease, and multiple sclerosis. The study of proteolipids is an active area of research in biochemistry and cell biology, with potential implications for the development of new therapies for neurological disorders.

2001). "Compact myelin dictates the differential targeting of two sodium channel isoforms in the same axon". Neuron. 30 (1): 91 ... In the internodal region, the Schwann cell has an outer collar of cytoplasm, a compact myelin sheath, and inner collar of ... The structure of the node and the flanking paranodal regions are distinct from the internodes under the compact myelin sheath, ... The internodal glial membranes are fused to form compact myelin, whereas the cytoplasm-filled paranodal loops of myelinating ...
Together they form and compact myelin sheaths of nerve cells. Plaque proteins are molecules, that are required for the ... PMP22/gas-3, called peripheral myelin protein, is located in the myelin sheath. The expression of this protein is associated ... an establishment of tight junction in the Schwamm cell membrane or a compact formation of myelin. It is also present in ... OSP/Claudin 11 is occurred in a myelin of nerve cells and between Sertoli cells, so it forms tight junctions in the CNS. This ...
Boiko T, Rasband MN, Levinson SR, Caldwell JH, Mandel G, Trimmer JS, Matthews G (April 2001). "Compact myelin dictates the ...
... of total protein content in compact myelin. Compact myelin is the bulk of the peripheral neuron's myelin sheath, a protective ... Peripheral myelin protein 22 has been shown to interact with myelin protein zero. GRCh38: Ensembl release 89: ENSG00000109099 ... it plays an essential role in the formation and maintenance of compact myelin. When Schwann cells come into contact with a ... Growth arrest-specific protein 3 (GAS-3), also called peripheral myelin protein 22 (PMP22), is a protein which in humans is ...
... is the connection of the outer cell membrane to the compact myelin sheath. The inner mesaxon (Terminologia histologica: Mesaxon ... 3-8, doi:10.1016/b978-0-7216-9491-7.50004-1, ISBN 978-0-7216-9491-7 Morell, Pierre; Quarles, Richard H. (1999). "The Myelin ... internum) is the connection between the myelin sheath and the inner part of the cell membrane of the Schwann cell, which is ...
Secondary incisures appear later, in regions of a compact myelin sheath, initially traversing only part of its radial thickness ... Myelin incisures (also known as Schmidt-Lanterman clefts, Schmidt-Lanterman incisures, clefts of Schmidt-Lanterman, segments of ... They are histological evidence of the small amount of cytoplasm that remains in the inner layer of the myelin sheath created by ... Primary incisures appear ab initio in myelination and always extend across the whole radial thickness of the myelin sheath but ...
... non-redundant role in formation of compact myelin; myelin oligodendrocyte glycoprotein (MOG), which is specific to the CNS; and ... Cholesterol is an essential lipid component of myelin, without which myelin fails to form. The main purpose of myelin is to ... Lesional demyelinations of the central nervous system Myelin-associated glycoprotein Myelin incisure The Myelin Project, ... These glial wraps are quite different from vertebrate compact myelin, formed, as indicated above, by concentric wrapping of the ...
Myelin is a highly ordered structure, in which many lipid- enriched, densely compacted phospholipid bilayers are spirally ... Bélanger, E.; Bégin, S.; Laffray, S.; De Koninck, Y.; Vallée, R.; Côté, D. (2009). "Quantitative myelin imaging with coherent ... The linear acyl chains of the phospholipid molecules present a perpendicular orientation with respect to the myelin surface. ... Possible biomedical-oriented applications of this technique are related to the study of the myelin and myelopathies. ...
... which is mainly responsible for the formation of the sheath of compact myelin. Although the detailed functions and mechanisms ... Epithelial membrane protein 3 (EMP3) is a trans-membrane signaling molecule that is encoded by the myelin-related gene EMP3. ... EMP3 is a member of the peripheral myelin protein gene family 22-kDa (PMP22), ... a myelin-related gene located in the critical 19q13.3 region, is epigenetically silenced and exhibits features of a candidate ...
In addition, this proteolipid is localized in compact myelin of cells in the nervous system and has been implicated in myelin ... "Rafts in adult peripheral nerve myelin contain major structural myelin proteins and myelin and lymphocyte protein (MAL) and ... Myelin and lymphocyte protein is a protein that in humans is encoded by the MAL gene. The protein encoded by this gene is a ... Frank M, Schaeren-Wiemers N, Schneider R, Schwab ME (Aug 1999). "Developmental expression pattern of the myelin proteolipid MAL ...
... resulting in the failure of oligodendrocytes to form compact myelin in the central nervous system. Axons in shiverer mice fail ... Molineaux SM, Engh H, de Ferra F, Hudson L, Lazzarini RA (1986). "Recombination within the myelin basic protein gene created ... They are also used to study myelin defects more generally in demyelinating diseases such as multiple sclerosis. The shiverer ... and myelin deficient (shi mld)". Behav Genet. 20 (2): 213-234. doi:10.1007/BF01067791. PMID 1693848. S2CID 686238. Baker M (12 ...
... myelin protein zero helps keep these sheets compact by serving as a "glue" that keeps the layers of myelin together. It does so ... Mutations to myelin protein zero can lead to issues with the development of myelin early on in life or myelin degeneration on ... Myelin protein zero holds together the myelin sheath by participating in homotypic interactions with other myelin protein zero ... Mutations in myelin protein zero are known to cause myelin degeneration and neuropathy. Mutations that reduce myelin protein ...
Overall, the oligodendrocytes in myelin organoids demonstrate the ability to form compact myelin that wraps and organizes ... This protocol was able to generate organoids with compact myelin formation by day 160. Another protocol developed in 2019 ... Myelin structure and integrity is inherently hard to study in humans at a molecular level. MRI can shed light on myelin ... The myelin organoids derived from hPSC after the CRISPR correction of PLP1 point mutations generated myelin after 20 weeks in ...
P0 has been shown to be essential for the formation of compact myelin, as P0 null mutant (P0-) mice showed severely aberrant ... the resulting myelin layers were very thin and poorly compacted. Unexpectedly, P0- mice also showed degeneration of both axons ... 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 between each coil. The inner ...
For many neuron fibers, a myelin sheath, rich in cholesterol since it is derived from compacted layers of Schwann cell or ... Demyelination (loss of myelin) is believed to be part of the basis for multiple sclerosis. Cholesterol binds to and affects the ...
Different lipids allow for different ranges of motion and can be compacted different.[citation needed] SP-B plays a role in ... Effectively, SP-B cuts and pastes pieces of the lipid bilayers to form the three dimensional structure of the tubular myelin. ... The lamellar bodies are then secreted into the fluid lining the interior of alveoli, and become tubular myelin. This role is ... Lamellar bodies are groups of lipids and protein that are structurally similar to tubular myelin, but are found inside instead ...
The CerS2 gene is compact in size and is located in a chromosomal region that is replicated early in the cell cycle. CerS2 ... suggesting that it is important for the synthesis of myelin sphingolipids. The lack of CerS2, as shown in knockout mice, ...
At moderate pH in the absence of Ca2+ pGSN is compact and globular. Low pH or the presence of >nM Ca2+ is associated with an ... It has been found in brain, testes, and lung oligodendrocytes, and is reportedly involved in myelin remodeling during ...
... s containing a sulfuric ester (sulfate) group, known as sulfatides, also occur in the myelin sheath of nerves. These ... These hydrogen bonds within the cerebrosides result in the molecules having a high transition temperature and compact alignment ... Cerebroside synthesis can therefore give a measurement of myelin formation or remyelination. The sugar moiety is linked ... Monogalactosylceramide is the largest single component of the myelin sheath of nerves. ...
How the fatty myelin sheaths of peripheral nerves were formed was a matter of debate that could not be answered until the ... cells that are independent and separate, e.g. blood cells cells that are independent but compacted together in layers, e.g. ...
... which may be caused by duplication of the gene encoding peripheral myelin protein 22 (PMP22) on chromosome 17. Inversions: A ... in the latter part of the process as haploid spermatids undergo major nuclear chromatin remodeling into highly compacted sperm ...
Myelination is arrested at the promyelinating stage and in fish no myelin basic protein can be detected. In fish this can be ... While the Fugu genome is relatively compact and limited with the number of adhesion GPCRs, the Tetraodon nigroviridis, another ... rescued by adding forskolin during development, which rescues myelin basic protein expression. GPR56 may play a role in the ...
Demyelination is the act of demyelinating, or the loss of the myelin sheath insulating the nerves. When myelin degrades, ... The soma is a compact structure, and the axon and dendrites are filaments extruding from the soma. Dendrites typically branch ... The myelin sheath in peripheral nerves normally runs along the axon in sections about 1 mm long, punctuated by unsheathed nodes ... To minimize metabolic expense while maintaining rapid conduction, many neurons have insulating sheaths of myelin around their ...
It affects only boys between the ages of 5 and 10 and destroys the protective cell surrounding the nerves, myelin, in the brain ... The partial inactivation of the X-chromosome is due to repressive heterochromatin that compacts the DNA and prevents the ...
Levels of myelin basic protein were elevated in his cerebrospinal fluid, suggesting that demyelination was occurring. This link ... The extracellular region of CD46 contains four short consensus repeats of about 60 amino acids that fold into a compact beta- ... The molecular mimicry hypothesis, in which T cells are essentially confusing an HHV-6 viral protein with myelin basic protein, ... Early on in the development of this hypothesis (2002), Italian researchers used the HHV-6a variant along with bovine myelin ...
The battery utilises a compact 3D design, intertwining its electrodes to maximize its surface area while reducing its volume. ... New York researchers successfully cure leukodystrophy in mice by using skin cells to repair damaged myelin sheaths. This ... "Tiny Batteries: 3-D Printing Could Lead to Miniaturized Medical Implants, Compact Electronics, Tiny Robots". Science Daily. 18 ... the immune system of multiple sclerosis patients is reported to reduce their reactivity to myelin by 50 to 75 percent. A newly ...
In the Ca2+-free state, the helices that form the four EF-hands are collapsed in a compact orientation, and the central linker ... "Myelin-associated Glycoprotein". Molecule of the Month. RCSB PDB. July 2020. Archived from the original on 2010-05-29. ...
Van Essen, DC (January 23, 1997). "A tension-based theory of morphogenesis and compact wiring in the central nervous system". ... Proteins expressed in glial cells include astrocyte markers GFAP and S100B whereas myelin basic protein and the transcription ...
... are surrounded by a myelin sheath. The myelin sheath may be compact and spiralling around the axon or it may be non-compact and ... We add tunnels to represent, as it should be, the compact myelin because in fact much of the riddle lies in the darkness of ... The tunnels are like compact myelin. The AP at noR seems compressed and looks quite stationary. Its duration is the same as the ... The action potential is even eliminated when the compact myelin disappears consecutively over a tiny length of the axon [24] . ...
2001). "Compact myelin dictates the differential targeting of two sodium channel isoforms in the same axon". Neuron. 30 (1): 91 ... In the internodal region, the Schwann cell has an outer collar of cytoplasm, a compact myelin sheath, and inner collar of ... The structure of the node and the flanking paranodal regions are distinct from the internodes under the compact myelin sheath, ... The internodal glial membranes are fused to form compact myelin, whereas the cytoplasm-filled paranodal loops of myelinating ...
Most of the remaining myelin, although thin, was still densely compacted. The peripheral nerve myelination was without ... Most myelin sheaths were uniform in thickness around the periphery of the axons. Some axons had no myelin detectable by light ... Drawn boxes show minimal myelin sheath disintegration at points where the axoplasma is still adherent to the myelin (upper box ... Infrequently seen in peripheral nerve were cytoplasmic residues, which still were attached to the myelin, with some myelin ...
Because Caspr mutant mice have normal compact myelin but aberrant paranodes (Bhat et al., 2001), they provide an ideal system ... Boiko T, Rasband MN, Levinson SR, Caldwell JH, Mandel G, Trimmer JS, Matthews G ( 2001) Compact myelin dictates the ... Defects were similar to those reported previously; compact myelin sheaths appeared to be normal in cross sections (data not ... Previous studies of a dysmyelinating mutant suggested that compact myelin and/or paranodal junctions regulate the expression of ...
Proper expression of the peripheral myelin protein of 22 kDa (PMP22), an integral constituent of the compact myelin sheath, is ... Myelin purification. Myelin purification was performed as previously described55. In short, 6 dissected sciatic nerves of rats ... Guerrero-Valero, M., et al. Dysregulation of myelin synthesis and actomyosin function underlies aberrant myelin in CMT4B1 ... Fixed cells were stained for myelin basic protein (MBP, grey/green) to visualize myelin and beta tubulin III (TUJ1, magenta) ...
Mutations of the CX32 gene that are not expressed in compact myelin and do not carry the L2/HNK-1 epitope are not associated ... Myelin genes and products affected in this manner include myelin protein zero (MPZ, P0), peripheral myelin protein 22 (PMP22), ... This is the case for myelin protein zero, PMP22, and myelin-associated glycoprotein; myelin protein zero may be a binding ... Interestingly, several conditions in which tomaculae occur result from either antibodies binding to proteins in compact myelin ...
... of the mass of CNS white matter and are believed to serve an important structural function in compact myelin. [2, 3, 4, 5, 6] ... Axonal swellings and degeneration in mice lacking the major proteolipid of myelin. Science. 1998 Jun 5. 280(5369):1610-3. [QxMD ... Thus, oligodendrocyte numbers are severely reduced, and little (if any) myelin is made. ...
Moreover, compact myelin sheaths are dense structures, composed of lipids (Salzer, 2015), and could have biophysical properties ... Hypervascularization of the sciatic nerve following inhibition of myelin production. To decipher the role of the myelin sheath ... Non-myelinating SC associate with multiple small caliber axons to form Remak bundles without forming compact myelin sheaths. ... Another question raised by our findings is do SC and myelin have a role in the maintenance of the INV? Since Wnt1-Cre;Krox20GFP ...
MBP to compact myelin (Sternberger et al., 1978), and MBP mRNA along the myelin internode (Colman et al., 1982; Trapp et al., ... Oligodendrocytes that ensheathed axons expressed and selectively targeted proteolipid protein to compact myelin and did not ... The myelin proteolipid protein gene and its expression. In Myelin: Biology and Chemistry. R.E. Martenson, editor. CRC Press, ... The myelin proteolipid protein gene and its expression. In Myelin: Biology and Chemistry. R.E. Martenson, editor. CRC Press, ...
... the vacuoles were occasionally formed on the sciatic nerve myelin sheath, with more compact and tidy layer arrangement compared ... Electron microscopy showed the intact structure of the myelin sheaths of the sciatic nerve fibers in Group C, whereas the ... structure of the nerve fibers was damaged, with a large number of vacuoles in the myelin sheath in Group A. In Group B, ...
... which are covered by compact myelin (Fig. 1). In the peripheral nervous system (PNS), this intricate axonal organization ... where the terminal loops of the myelin attach to the axon; (3) the juxtaparanodal region, where Kv1 potassium channels are ... which are gaps between myelin segments where sodium channels are clustered; (2) the paranodal axoglial junction, ...
... compact myelin. Experimental Neurology 291: 74-86 (2017). *Beltran. Reversing Paralysis: Stem cell therapy aims to repair ... Astrocytes provide an environment for the normal function of nerve cells, while oligodendrocytes form the myelin that surrounds ...
... function attributed to the myelin transmembrane protein in maintaining the multilamellar, compact architecture of myelin. On ... We have explored the interaction of the Myelin Proteolipid with a nonionic lamellar (L. ) phase. In contrast to water-soluble ... OIL-SWOLLEN BILAYERS BY A MYELIN TRANSMEMBRANE PROTEIN AND BY SYNTHETIC PEPTIDES. N. Taulier, C. Nicot, M. Waks, LIP, CNRS 7623 ...
... of the mass of CNS white matter and are believed to serve an important structural function in compact myelin. [2, 3, 4, 5, 6] ... Axonal swellings and degeneration in mice lacking the major proteolipid of myelin. Science. 1998 Jun 5. 280(5369):1610-3. [QxMD ... Thus, oligodendrocyte numbers are severely reduced, and little (if any) myelin is made. ...
compact myelin + concave side of sperm head cone matrix sheath conoid contractile ring + ...
At 4 months of age, NMR brains reach 90% of adult size with stable neuronal cytostructural protein expression whereas myelin ... of adult size with stable neuronal cytostructural protein expression whereas myelin protein expression does not plateau until ... MBP, however, begins to compact the cytoplasmic leaflets of the myelin bilayer only after myelin has been produced, and ... myelin-associated glycoprotein; MBP, myelin basic protein; NF, neurofilament; NF-L, neurofilament light chain; TH, tyrosine ...
The C-terminal tail of P0 adheres apposing membranes together in the narrow cytoplasmic compartment of compact myelin, much ... Therefore, P0 is the executive molecule of PNS myelin maturation. How and when is P0 trafficked and modified to enable myelin ... Myelin protein zero (P0), a type I transmembrane protein, is the most abundant protein in peripheral nervous system (PNS) ... Subject: Biology And Life Sciences, Cell And Developmental Biology Keywords: myelin; myelination; development; peripheral ...
non-compact myelin associated.... A930040G15Rik. 84626. KRBA1. KRAB-A domain containing 1 [S.... ...
PMP22 is produced mainly in Schwann cells in the peripheral nervous system where it is localised to compact myelin. The ... The mechanism of neuropathy in peripheral myelin protein 22 mutant mice. Doctoral thesis (Ph.D), UCL (University College London ... The mechanism of neuropathy in peripheral myelin protein 22 mutant mice. Open access status:. An open access version is ... In C22 (7 copy) animals myelin formation was delayed or non-existent in many fibres whereas in C61 animals myelination ...
Myelin is the tightly compacted multilamellar sheath that surrounds axons and promotes saltatory conduction of nerve impulses. ... particularly with regard to the formation and maintenance of myelin. ... particularly with regard to the formation and maintenance of myelin. Myelin is the tightly compacted multilamellar sheath that ... The myelin proteolipid protein gene (PLP1) encodes the most abundant protein found in mature myelin from the CNS. Expression of ...
E-Cadherin is not found, however, in compact myelin bilayers. Rather, it is concentrated at the paranodes, in Schmidt-Lanterman ...
For example, the degradation of myelin, which forms a compact lipid-rich sheath around neuronal axons to facilitate conduction ... This indicates that chronic and acute myelin dysfunction trigger amyloid deposition and reveals myelin defects as a risk factor ... crossed with mice showing minor structural myelin defects driven by the lack of myelin-proteins like CNP (CNP-/-) or PLP (PLP-/ ... 2) Would it be possible to change the focus of microglia once they are distracted by myelin debris and urge them to react to ...
2008 The antibodies used and their sources were: SMI-94R (Covance Princeton NJ U.S.A.); compact myelin protein zero (P0) ... myelin basic protein). Degenerated myelin segments were quantified Rabbit polyclonal to RAB27A. as previously explained (Yu et ... The cultures were co-stained for MBP and PGP9.5 and nuclei were visualized with DAPI (4′ 6 Degeneration of the myelin segments ... was quantified with the aid of the open source imaging software Cell Profiler (http://www.cellprofiler.org). Individual myelin ...
... a biochemical assay for PMP22 function and evidence that PMP22 directly contributes to membrane organization in compact myelin. ... Peripheral myelin protein 22 alters membrane architecture. by Kathleen F. Mittendorf; Justin T. Marinko; Cheri M. Hampton; ... Peripheral myelin protein 22 (PMP22) is highly expressed in myelinating Schwann cells of the peripheral nervous system. PMP22 ... Formation of the myelin-like assemblies is disrupted by a CMTD-causing mutation. This study provides both ...
... which enwrap axons with several layers of compacted membrane. The axon/myelin-unit is often impaired in myelin-related ... many of them localized to its non-compacted sub-compartments. For example, one of the latest steps of myelin maturation in the ... Interestingly, these myelin outfoldings are a very specific neuropathology, as other analyzed neuropathological features were ... The results lead to the assumption that anillin-dependent scaffolding of mature myelin by septin filaments is required for its ...
compact myelin concave side of sperm head cone matrix sheath contractile ring ...
... and axons in the optic fiber layer are wrapped with compact myelin within the retina; however, the expression of myelin genes ... and axons in the optic fiber layer are wrapped with compact myelin within the retina; however, the expression of myelin genes ... and axons in the optic fiber layer are wrapped with compact myelin within the retina; however, the expression of myelin genes ... and axons in the optic fiber layer are wrapped with compact myelin within the retina; however, the expression of myelin genes ...
  • All axons (with very, very rare exceptions) are surrounded by a myelin sheath. (scirp.org)
  • The myelin sheath may be compact and spiralling around the axon or it may be non-compact and form a simple envelope around the axon. (scirp.org)
  • In neuroscience and anatomy, nodes of Ranvier (/ˈrɑːnvieɪ/ RAHN-vee-ay), also known as myelin-sheath gaps, occur along a myelinated axon where the axolemma is exposed to the extracellular space. (wikipedia.org)
  • Dendrite Soma Axon Nucleus Node of Ranvier Axon terminal Schwann cell Myelin sheath Many vertebrate axons are surrounded by a myelin sheath, allowing rapid and efficient saltatory ("jumping") propagation of action potentials. (wikipedia.org)
  • The structure of the node and the flanking paranodal regions are distinct from the internodes under the compact myelin sheath, but are very similar in CNS and PNS. (wikipedia.org)
  • In the internodal region, the Schwann cell has an outer collar of cytoplasm, a compact myelin sheath, and inner collar of cytoplasm, and the axolemma. (wikipedia.org)
  • Proper expression of the peripheral myelin protein of 22 kDa (PMP22), an integral constituent of the compact myelin sheath, is important for development and function of peripheral nerve fibers. (biorxiv.org)
  • Myelinating Schwann cells form a myelin sheath around a single axon and express high levels of myelin-related proteins and messenger RNA (mRNA). (medscape.com)
  • The portion of the myelin sheath in which layers of cell membrane are tightly juxtaposed, completely excluding cytoplasm. (zfin.org)
  • Electron microscopy showed the intact structure of the myelin sheaths of the sciatic nerve fibers in Group C, whereas the structure of the nerve fibers was damaged, with a large number of vacuoles in the myelin sheath in Group A. In Group B, the vacuoles were occasionally formed on the sciatic nerve myelin sheath, with more compact and tidy layer arrangement compared with Group A. (nih.gov)
  • Myelin is the tightly compacted multilamellar sheath that surrounds axons and promotes saltatory conduction of nerve impulses. (uams.edu)
  • For example, the degradation of myelin, which forms a compact lipid-rich sheath around neuronal axons to facilitate conduction speed, is markedly increased in the aging human brain (3). (biologists.com)
  • A myelin sheath, which is rich in cholesterol since it is derived from compacted layers of Schwann cell membrane, provides protection for many neuron fibres, allowing for more effective impulse conduction. (vedantu.com)
  • Schwann cells wrap myelin around peripheral nerve axons for fast neural transmission 1 . (biorxiv.org)
  • PMP22 is produced mainly in Schwann cells in the peripheral nervous system where it is localised to compact myelin. (ucl.ac.uk)
  • Results We 1st record that myelin oligodendrocytes and Schwann cells communicate Compact disc47 without SIRPα and additional concur that microglia and macrophages communicate both Compact disc47 and SIRPα. (bioshockinfinitereleasedate.com)
  • It may further be argued that CD47 functions RAF265 normally as a marker of "self" that helps protect intact myelin and myelin-forming oligodendrocytes and Schwann cells from activated microglia and macrophages. (bioshockinfinitereleasedate.com)
  • History Oligodendrocytes in RAF265 the central anxious program (CNS) and Schwann cells in the peripheral anxious system (PNS) type specific myelin extensions of their plasma membranes that surround axons normally allowing them fast conduction of electric activity. (bioshockinfinitereleasedate.com)
  • 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)
  • This is not surprising given the strong evidence for interaction between myelin and axon gene expression in development and after experimental nerve lesions. (medscape.com)
  • The axon/myelin-unit is often impaired in myelin-related disorders and upon normal aging. (uni-goettingen.de)
  • Duplication of the gene encoding the myelin protein PMP22 causes the hereditary neuropathy Charcot-Marie-Tooth disease 1A (CMT1A), characterized by hypomyelination of medium to large caliber peripheral axons. (biorxiv.org)
  • A hallmark of the HNPP disease is the formation of tomacula, extensive formation of myelin sheaths at cytoplasmic areas, such as paranodes and Schmidt-Lanterman incisures leading to deformed and constricted axons and subsequently demyelination 7 - 9 . (biorxiv.org)
  • Oligodendrocytes that ensheathed axons expressed and selectively targeted proteolipid protein to compact myelin and did not degenerate. (rupress.org)
  • Astrocytes provide an environment for the normal function of nerve cells, while oligodendrocytes form the myelin that surrounds the axons of nerve cells, aiding electrical signaling. (reprocell.com)
  • In the central nervous system (CNS), myelin is formed by oligodendrocytes, which enwrap axons with several layers of compacted membrane. (uni-goettingen.de)
  • Background Traumatic injury to axons produces break down of axons and myelin in the site from the lesion and further distal to the where Wallerian degeneration develops. (bioshockinfinitereleasedate.com)
  • Distressing problems for axons produces abrupt break down of myelin and axons where physical trauma occurs. (bioshockinfinitereleasedate.com)
  • After that axons and myelin also breakdown distal towards the lesion as Wallerian degeneration (WD) builds up [1]. (bioshockinfinitereleasedate.com)
  • At 4 months of age, NMR brains reach 90% of adult size with stable neuronal cytostructural protein expression whereas myelin protein expression does not plateau until 9 months of age in NMRs, and synaptic protein expression continues to change throughout the first 3 years of life. (frontiersin.org)
  • Mutations in the gene for peripheral myelin protein 22 (PMP22) are associated with peripheral neuropathy in mice and humans. (ucl.ac.uk)
  • The function of PMP22 is unclear but its low abundance makes it unlikely to be a structural myelin protein. (ucl.ac.uk)
  • The myelin proteolipid protein gene (PLP1) encodes the most abundant protein found in mature myelin from the CNS. (uams.edu)
  • R&D Systems Minneapolis MN U.S.A.). After 48 h the cultures were fixed and stained for MBP (myelin basic protein). (ecolowood.com)
  • Mutations in the compact myelin protein gene pmp22 cause more than 50% of all CMTs. (isciii.es)
  • In my presentation, I will summarize recent QENS and NSE results on the dynamics of the intrinsically disordered myelin basic protein (MBP) and the chemically denatured bovine serum albumin (BSA) (1,2,3). (lu.se)
  • Oligodendrocytes, the myelin-forming cell in the central nervous system (CNS) 1 , are neuroepithelial in origin. (rupress.org)
  • We used a novel mouse model (oLucR) expressing luciferase in myelin oligodendrocyte glycoprotein-positive oligodendrocytes under the control of a β-actin promoter. (biomedcentral.com)
  • [ 6 ] However, the gene mutations responsible for the different forms of CMT1 are clearly myelin genes. (medscape.com)
  • Thus, oligodendrocyte numbers are severely reduced, and little (if any) myelin is made. (medscape.com)
  • 2D2 mice, which exhibit a transgenic T cell receptor (TCR) particular for myelin-oligodendrocyte glycoprotein (MOG35-55), had been bred at Victoria School of Wellington (Wellington, New Zealand). (healthandwellnesssource.org)
  • However, only little is known about proteins that maintain the structure of mature myelin, many of them localized to its non-compacted sub-compartments. (uni-goettingen.de)
  • The results lead to the assumption that anillin-dependent scaffolding of mature myelin by septin filaments is required for its normal function in facilitating rapid nerve conduction in the healthy CNS. (uni-goettingen.de)
  • Conversely, haplo-insufficiency of PMP22 leads to focal myelin overgrowth in hereditary neuropathy with liability to pressure palsies (HNPP). (biorxiv.org)
  • However, the molecular mechanisms of myelin growth regulation by PMP22 remain obscure. (biorxiv.org)
  • Ionic environment, thickness and line tension as determinants of phase separation in whole Purified Myelin Membranes monolayers. (tu-darmstadt.de)
  • Consequently, the authors of this preprint hypothesized that defective myelin prevents microglia from clearing Aβ, thus contributing to the development of AD. (biologists.com)
  • Down-regulation which we check here comes after binding of myelin Compact disc47 towards the immune system inhibitory receptor SIRPα (sign regulatory proteins-α) on macrophages and microglia. (bioshockinfinitereleasedate.com)
  • Degenerated myelin is certainly phagocytosed at damage sites in traumatized CNS by turned on resident microglia and by turned on blood-borne macrophages that access the website through ruptured vasculature. (bioshockinfinitereleasedate.com)
  • On the other hand macrophages aren't recruited and resident microglia aren't turned on to phagocytose degenerated myelin distal to lesion where CNS-WD builds up [2-4]. (bioshockinfinitereleasedate.com)
  • The usage of healthful mice being a source of principal immune system cells (bone tissue marrow-derived macrophages, microglia, and Compact disc4 T cells) was accepted by the pet Ethics Committee of Victoria School of Wellington (2011-R21). (healthandwellnesssource.org)
  • The internodes are the myelin segments and the gaps between are referred to as nodes. (wikipedia.org)
  • The cultures were co-stained for MBP and PGP9.5 and nuclei were visualized with DAPI (4′ 6 Degeneration of the myelin segments was quantified with the aid of the open source imaging software Cell Profiler (http://www.cellprofiler.org). (ecolowood.com)
  • Individual myelin internodes were recognized using Otsu's method for thresholding and segmentation (Otsu 1979 Segmentation was visually inspected for errors or regions where segments were closely. (ecolowood.com)
  • These proteins constitute about 50% of the mass of CNS white matter and are believed to serve an important structural function in compact myelin. (medscape.com)
  • assessed amyloid deposition in AD mouse models (5xFAD and APP NLGF , respectively) crossed with mice showing minor structural myelin defects driven by the lack of myelin-proteins like CNP (CNP -/- ) or PLP (PLP -/y ). 5XFAD mice harbor five familial AD (FAD) mutations triggering Aβ overproduction, and already exhibit amyloid deposition two months after birth (4). (biologists.com)
  • Within this model, Compact disc4+ IFN- and T-cells had been essential for disease advancement (3, 4), with nearly all pathogenic effectors particularly recognizing myelin proteins 0 (5). (ubatubasat.com)
  • These membrane domains remain directly connected to the cell body with a complex underlying cytoarchitecture comprising microtubules distributed in larger processes and actin filaments enriched in thinner myelin domains and in paranodes [ 6 , 7 ]. (biomedcentral.com)
  • By focused ion beam-scanning electron microscopy (FIB-SEM), it has been observed that these outfoldings are large sheets of multiple compact membrane layers, a plausible explanation for an altered membrane resistance along myelinated fiber tracts and thus, the observed reduced nerve conduction velocity. (uni-goettingen.de)
  • Nerve and islet infiltrating Compact disc4+ T-cells differed by appearance patterns of Compact disc95 also, Tim-3 and PD-1. (ubatubasat.com)
  • however, the expression of myelin genes in the chick retina has not been demonstrated yet. (elsevierpure.com)
  • In situ hybridization demonstrated that all three myelin genes began to be expressed at E14 in the chick embryo retina. (elsevierpure.com)
  • The present results elucidate that myelin genes are expressed only by OLs that are mostly localized in the innermost layer of the developing chick retina. (elsevierpure.com)
  • This conduction is indeed very different and faster than for fibres "without" myelin. (scirp.org)
  • Finally, IL-2 blockade in NOD mice, furthermore to exacerbating T1D advancement, triggers the forming of multi-system autoimmunity, including peripheral neuropathy mediated both by Compact disc4+ also to a lesser level, Compact disc4? (ubatubasat.com)
  • Therefore myelin-associated inhibitory substances (e.g. (bioshockinfinitereleasedate.com)
  • Conversely, type II macrophages (generated through arousal with LPS and immune system complexes, IC) generate higher degrees of IL-10, and lower degrees of IL-12 and many co-stimulatory/inhibitory molecules such as for example CD40, Compact disc86, Compact disc80 and PD-L1 [3]. (healthandwellnesssource.org)
  • To further investigate whether myelin defects can cause plaque deposition, they induced acute demyelination in 5xFAD mice by feeding them Cuprizone. (biologists.com)
  • This indicates that chronic and acute myelin dysfunction trigger amyloid deposition and reveals myelin defects as a risk factor for plaque formation. (biologists.com)
  • To investigate the impact of impaired myelin on AD pathogenesis, they used various mouse models with genetically or chemically induced myelin dysfunction. (biologists.com)
  • Here using toxic and genetic mouse models of demyelination and dysmyelination, respectively, we find that loss of compact myelin reduces PV+ interneuron presynaptic terminals, increases failures and the weak phasic inhibition of pyramidal neurons abolishes optogenetically driven gamma oscillations in vivo. (nin.nl)
  • Similarly, induction of experimental autoimmune encephalomyelitis (EAE) - a model for chronic immune-mediated myelin loss - caused the formation of amyloid plaques in the spinal cord of 5xFAD mice. (biologists.com)
  • In 6-month-old CNP-/-5xFAD mice, they observed increased expression of BACE1 accompanied by elevated levels of APP fragments, which suggests enhanced Aβ generation driven by defective myelin. (biologists.com)
  • By quantitative mass spectrometry (MS) analysis of purified myelin and immunoblot validation, a specific reduction of ANLN, SEPT2, SEPT4, SEPT7, and SEPT8 has been detected in Anln cKO mice compared to controls. (uni-goettingen.de)
  • Interestingly, these myelin outfoldings are a very specific neuropathology, as other analyzed neuropathological features were not observed in Anln cKO mice. (uni-goettingen.de)
  • investigated the impact of severe reduction of cortical myelin on amyloid pathology. (biologists.com)
  • Electron micrograph analysis revealed the emergence of pathological myelin outfoldings upon conditional ANLN deletion and the observed reduction of myelin septins. (uni-goettingen.de)
  • Through mechanisms that at present can only be speculated about, myelin disturbances result in axonal damage. (medscape.com)
  • Also, the fact that ODCs close to or within neuroinflammatory lesions that have been deprived of their myelin processes can survive this insult [ 25 , 26 ] suggests the existence of active mechanisms of cellular plasticity. (biomedcentral.com)
  • Antibody depletion research demonstrated neuritis created in the lack of NOD-CD8+ T-cells still, but required Compact disc4+ T-cells. (ubatubasat.com)
  • Finally, we discovered that another type II-activating substance, glatiramer acetate, Dooku1 didn't bias Compact disc4+ T cells to create enhanced IL-17A. (healthandwellnesssource.org)
  • Prior studies show that while traditional macrophages bias na also?ve Compact disc4+ T cells toward a Th1 phenotype, type II macrophages get Compact disc4+ T cells towards a Th2 response [4] and [3]. (healthandwellnesssource.org)
  • For example, when Compact disc40 and IL-23 aren't portrayed by cells within the CNS, the severe Dooku1 nature of disease is normally reduced in experimental autoimmune encephalomyelitis (EAE) an pet style of multiple sclerosis (MS) [6, 7]. (healthandwellnesssource.org)
  • The focus of research in my laboratory is centered on CNS development, particularly with regard to the formation and maintenance of myelin. (uams.edu)
  • Moreover, they speculated that loss of myelin integrity triggers the formation of Aβ plaques. (biologists.com)
  • For example, one of the latest steps of myelin maturation in the CNS is the assembly of septin filaments, which prevent the formation of pathological myelin outfoldings. (uni-goettingen.de)
  • Animal myelin transcription factor 1 (MyT1), or neural zinc finger 2 (NZF2), a transcription factor that contains seven copies of the CCHHC-type zinc finger. (embl.de)
  • T cell infiltration from the central anxious program, and inhibited systemic Compact disc4+ T cell replies to myelin epitopes. (biobender.com)
  • Here, we found that the major inhibitor of the myelin growth signaling pathway PI3K/Akt/mTOR, phosphatase and tensin homolog (PTEN) is increased in abundance in CMT1A and decreased in HNPP rodent models. (biorxiv.org)
  • Unexpectedly phagocytosis of CD47-/- myelin Sav1 by SIRPα-KD phagocytes which is not altered from normal when tested in serum-free medium RAF265 is usually augmented when serum is present. (bioshockinfinitereleasedate.com)
  • In support of a causal role of impaired PV-mediated inhibition, optogenetic activation of myelin-deficient PV+ interneurons attenuated the power of slow theta rhythms and limited interictal spike occurrence. (nin.nl)
  • Purified myelin lipids display a critical mixing point at low surface pressure. (tu-darmstadt.de)