Myelin Regulatory Factor
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Myelin Regulatory Factor
Myelin regulatory factor (MRF), Myelin gene regulatory factor, is a protein that in humans is encoded by the ''MYRF'' gene. Orthologs Myelin regulatory factor is encoded by the ''Myrf/GM98'' gene in mice and by the ''MYRF'' gene in humans. The family of MyRF-like-proteins also contains the orthologues pqn-47 from ''Caenorhabditis elegans, C. elegans'' and MYRFA from ''Dictyostelium''. All orthologs have a DNA-binding domain of high homology to the ''Saccharomyces cerevisiae'' protein Ndt80 (a p53-like transcription factor) and therefore likely act as a transcription factor. Function MyRF is a transcription factor that promotes the expression of many genes important in the production of myelin. It is therefore of critical importance in the development and maintenance of myelin sheaths. The expression of MYRF is specific to mature, myelinating oligodendrocytes in the central nervous system, CNS. It has been shown to be critical for the maintenance of myelin by these cell ...
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Protein
Proteins are large biomolecules and macromolecules that comprise one or more long chains of amino acid residue (biochemistry), residues. Proteins perform a vast array of functions within organisms, including Enzyme catalysis, catalysing metabolic reactions, DNA replication, Cell signaling, responding to stimuli, providing Cytoskeleton, structure to cells and Fibrous protein, organisms, and Intracellular transport, transporting molecules from one location to another. Proteins differ from one another primarily in their sequence of amino acids, which is dictated by the Nucleic acid sequence, nucleotide sequence of their genes, and which usually results in protein folding into a specific Protein structure, 3D structure that determines its activity. A linear chain of amino acid residues is called a polypeptide. A protein contains at least one long polypeptide. Short polypeptides, containing less than 20–30 residues, are rarely considered to be proteins and are commonly called pep ...
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Ablation
Ablation ( – removal) is the removal or destruction of something from an object by vaporization, chipping, erosion, erosive processes, or by other means. Examples of ablative materials are described below, including spacecraft material for ascent and atmospheric reentry, ice and snow in glaciology, biological tissues in medicine and passive fire protection materials. Artificial intelligence In artificial intelligence (AI), especially machine learning, Ablation (artificial intelligence), ablation is the removal of a component of an AI system. The term is by analogy with biology: removal of components of an organism. Biology Biological ablation is the removal of a biological structure or functionality. Genetic ablation is another term for gene silencing, in which gene expression is abolished through the alteration or deletion of genetic sequence information. In cell ablation, individual cells in a population or culture are destroyed or removed. Both can be used as experimen ...
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NPC1
Niemann-Pick disease, type C1 (NPC1) is a membrane protein that mediates intracellular cholesterol trafficking in mammals. In humans the protein is encoded by the ''NPC1'' gene (chromosome location 18q11). Function ''NPC1'' was identified as the gene that when mutated, results in Niemann-Pick disease, type C. Niemann-Pick disease, type C is a rare neurovisceral lipid storage disorder resulting from autosomal recessively inherited loss-of-function mutations in either ''NPC1'' or ''NPC2''. This disrupts intracellular lipid transport, leading to the accumulation of lipid products in the late endosomes and lysosomes. Approximately 95% of NPC patients are found to have mutations in the ''NPC1'' gene. ''NPC1'' encodes a putative integral membrane protein containing sequence motifs consistent with a role in intracellular transport of cholesterol and sphingosine to post- lysosomal destinations. Clinical significance Obesity Mutations in the ''NPC1'' gene have been strongl ...
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Oligodendrocyte
Oligodendrocytes (), also known as oligodendroglia, are a type of neuroglia whose main function is to provide the myelin sheath to neuronal axons in the central nervous system (CNS). Myelination gives metabolic support to, and insulates the axons of most vertebrates. A single oligodendrocyte can extend its Cellular extensions, processes to cover up to 40 axons, that can include multiple adjacent axons. The myelin sheath is segmented along the axon's length at gaps known as the nodes of Ranvier. In the peripheral nervous system the myelination of axons is carried out by Schwann cells. Oligodendrocytes are found exclusively in the CNS, which comprises the brain and spinal cord. They are the most widespread cell lineage, including oligodendrocyte progenitor cells, pre-myelinating cells, and mature myelinating oligodendrocytes in the CNS white matter. Non-myelinating oligodendrocytes are found in the grey matter surrounding and lying next to neuronal cell bodies. They are known as neu ...
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Myelin Sheath
Myelin Sheath ( ) is a lipid-rich material that in most vertebrates surrounds the axons of neurons to insulate them and increase the rate at which electrical impulses (called action potentials) pass along the axon. The myelinated axon can be likened to an electrical wire (the axon) with insulating material (myelin) around it. However, unlike the plastic covering on an electrical wire, myelin does not form a single long sheath over the entire length of the axon. Myelin ensheaths part of an axon known as an internodal segment, in multiple myelin layers of a tightly regulated internodal length. The ensheathed segments are separated at regular short unmyelinated intervals, called nodes of Ranvier. Each node of Ranvier is around one micrometre long. Nodes of Ranvier enable a much faster rate of conduction known as saltatory conduction where the action potential recharges at each node to jump over to the next node, and so on till it reaches the axon terminal. At the terminal the a ...
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Multiple Sclerosis
Multiple sclerosis (MS) is an autoimmune disease resulting in damage to myelinthe insulating covers of nerve cellsin the brain and spinal cord. As a demyelinating disease, MS disrupts the nervous system's ability to Action potential, transmit signals, resulting in a range of signs and symptoms, including physical, cognitive disability, mental, and sometimes psychiatric problems. Symptoms include double vision, vision loss, eye pain, muscle weakness, and loss of Sensation (psychology), sensation or coordination. MS takes several forms, with new symptoms either occurring in isolated attacks (relapsing forms) or building up over time (progressive forms). In relapsing forms of MS, symptoms may disappear completely between attacks, although some permanent neurological problems often remain, especially as the disease advances. In progressive forms of MS, bodily function slowly deteriorates once symptoms manifest and will steadily worsen if left untreated. While its cause is unclear, ...
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Myelin Oligodendrocyte Glycoprotein
Myelin oligodendrocyte glycoprotein (MOG) is a glycoprotein believed to be important in the myelination of nerves in the central nervous system (CNS). In humans this protein is encoded by the ''MOG'' gene. It is speculated to serve as a necessary "adhesion molecule" to provide structural integrity to the myelin sheath and is known to develop late on the oligodendrocyte. Molecular function While the primary molecular function of MOG is not yet known, its likely role with the myelin sheath is either in sheath "completion and/or maintenance". More specifically, MOG is speculated to be "necessary" as an "adhesion molecule" on the myelin sheath of the CNS to provide the structural integrity of the myelin sheath.Berger, T., Innsbruck Medical University Dept. of Neurology interviewed by S. Gillooly, Nov. 24, 2008." MOG's cDNA coding region in humans have been shown to be "highly homologous" to rats, mice, and bovine, and hence highly conserved. This suggests "an important biological ...
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Myelin-associated Glycoprotein
Myelin-associated glycoprotein (MAG), or Siglec-4 is a type 1 transmembrane protein, a glycoprotein localized in periaxonal Schwann cell and oligodendrocyte membranes, where it plays a role in glial-axonal interactions. MAG is a member of the SIGLEC family of proteins and is a functional ligand of the NOGO-66 receptor, NgR. MAG is believed to be involved in myelination during remyelination (nerve regeneration) in the peripheral nervous system (PNS) and is vital for the long-term survival of the myelinated axons following myelinogenesis. In the CNS MAG is one of three main myelin-associated inhibitors of axonal regeneration after injury, making it an important protein for future research on neurogenesis in the CNS. Structure MAG is a 100 kDA glycoprotein. Uncleaved MAG is a complete transmembrane form, which acts as a signaling and adhesion molecule. MAG can also act as a signaling molecule as a soluble protein after it has been proteolytically shed. This form of the prot ...
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Myelin Basic Protein
Myelin basic protein (MBP) is a protein important in the process of myelination of nerves in the nervous system. The myelin sheath is a multi-layered membrane, unique to the nervous system, that functions as an insulator to greatly increase the velocity of Action potential, axonal impulse conduction. MBP maintains the correct structure of myelin, interacting with the lipids in the myelin membrane. MBP was initially sequencing, sequenced in 1971 after isolation from bovine myelin cell membrane, membranes. MBP knockout mouse, knockout mice called Shiverer mouse, shiverer mice were subsequently developed and characterized in the early 1980s. ''Shiverer'' mice exhibit decreased amounts of CNS myelination and a progressive disorder characterized by tremors, seizures, and early death. The human gene for MBP is on chromosome 18; the protein localizes to the CNS and to various cell (biology), cells of the Haematopoiesis, hematopoietic lineage. The pool of MBP in the central nervous syste ...
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Proteolipid Protein 1
Proteolipid protein 1 (PLP1) is a form of myelin proteolipid protein (PLP). Mutations in ''PLP1'' are associated with Pelizaeus–Merzbacher disease. It is a 4 transmembrane domain protein which is proposed to bind other copies of itself on the extracellular side of the membrane. In a myelin sheath, as the layers of myelin wraps come together, PLP will bind itself and tightly hold the cellular membranes together. This gene encodes a transmembrane proteolipid protein that is the predominant myelin protein present in the central nervous system (CNS). The encoded protein functions in myelination. This protein may play a role in the compaction, stabilization, and maintenance of myelin sheaths, as well as in oligodendrocyte development and axonal survival. Mutations associated with this gene cause X-linked Pelizaeus–Merzbacher disease and spastic paraplegia type 2. Two transcript variants encoding distinct isoforms have been identified for this gene. In melanocytic cells PLP1 gene e ...
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Central Nervous System
The central nervous system (CNS) is the part of the nervous system consisting primarily of the brain, spinal cord and retina. The CNS is so named because the brain integrates the received information and coordinates and influences the activity of all parts of the bodies of bilateria, bilaterally symmetric and triploblastic animals—that is, all multicellular animals except sponges and Coelenterata, diploblasts. It is a structure composed of nervous tissue positioned along the Anatomical_terms_of_location#Rostral,_cranial,_and_caudal, rostral (nose end) to caudal (tail end) axis of the body and may have an enlarged section at the rostral end which is a brain. Only arthropods, cephalopods and vertebrates have a true brain, though precursor structures exist in onychophorans, gastropods and lancelets. The rest of this article exclusively discusses the vertebrate central nervous system, which is radically distinct from all other animals. Overview In vertebrates, the brain and spinal ...
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Gene
In biology, the word gene has two meanings. The Mendelian gene is a basic unit of heredity. The molecular gene is a sequence of nucleotides in DNA that is transcribed to produce a functional RNA. There are two types of molecular genes: protein-coding genes and non-coding genes. During gene expression (the synthesis of Gene product, RNA or protein from a gene), DNA is first transcription (biology), copied into RNA. RNA can be non-coding RNA, directly functional or be the intermediate protein biosynthesis, template for the synthesis of a protein. The transmission of genes to an organism's offspring, is the basis of the inheritance of phenotypic traits from one generation to the next. These genes make up different DNA sequences, together called a genotype, that is specific to every given individual, within the gene pool of the population (biology), population of a given species. The genotype, along with environmental and developmental factors, ultimately determines the phenotype ...
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