Neuroglial cells of the peripheral nervous system which form the insulating myelin sheaths of peripheral axons.
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 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.
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.
Nerve fibers that are capable of rapidly conducting impulses away from the neuron cell body.
Renewal or physiological repair of damaged nerve tissue.
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.
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)
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.
A moderately firm, benign, encapsulated tumor resulting from proliferation of SCHWANN CELLS and FIBROBLASTS that includes portions of nerve fibers. The tumors usually develop along peripheral or cranial nerves and are a central feature of NEUROFIBROMATOSIS 1, where they may occur intracranially or involve spinal roots. Pathologic features include fusiform enlargement of the involved nerve. Microscopic examination reveals a disorganized and loose cellular pattern with elongated nuclei intermixed with fibrous strands. (From Adams et al., Principles of Neurology, 6th ed, p1016)
An octamer transcription factor that plays an important role in the MYELIN SHEATH development by SCHWANN CELLS.
A peptide factor originally identified by its ability to stimulate the phosphorylation the erbB-2 receptor (RECEPTOR, ERBB-2). It is a ligand for the erbB-3 receptor (RECEPTOR, ERBB-3) and the erbB-4 receptor. Variant forms of NEUREGULIN-1 occur through alternative splicing of its mRNA.
Sensory ganglia located on the dorsal spinal roots within the vertebral column. The spinal ganglion cells are pseudounipolar. The single primary branch bifurcates sending a peripheral process to carry sensory information from the periphery and a central branch which relays that information to the spinal cord or brain.
MYELIN-specific proteins that play a structural or regulatory role in the genesis and maintenance of the lamellar MYELIN SHEATH structure.
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.
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.
A neoplasm that arises from SCHWANN CELLS of the cranial, peripheral, and autonomic nerves. Clinically, these tumors may present as a cranial neuropathy, abdominal or soft tissue mass, intracranial lesion, or with spinal cord compression. Histologically, these tumors are encapsulated, highly vascular, and composed of a homogenous pattern of biphasic fusiform-shaped cells that may have a palisaded appearance. (From DeVita Jr et al., Cancer: Principles and Practice of Oncology, 5th ed, pp964-5)
A family of peptides originally found as factors that stimulate the phosphorylation of the erbB-2 receptor (RECEPTORS, ERBB-2). Multiple variant forms of NEUREGULINS occur due to alternative splicing of their mRNAs. The NEUREGULINS include products from the three known genes (NGR1; NGR2 and NGR3).
Disease or damage involving the SCIATIC NERVE, which divides into the PERONEAL NERVE and TIBIAL NERVE (see also PERONEAL NEUROPATHIES and TIBIAL NEUROPATHY). Clinical manifestations may include SCIATICA or pain localized to the hip, PARESIS or PARALYSIS of posterior thigh muscles and muscles innervated by the peroneal and tibial nerves, and sensory loss involving the lateral and posterior thigh, posterior and lateral leg, and sole of the foot. The sciatic nerve may be affected by trauma; ISCHEMIA; COLLAGEN DISEASES; and other conditions. (From Adams et al., Principles of Neurology, 6th ed, p1363)
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.
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.
Treatment of muscles and nerves under pressure as a result of crush injuries.
A factor identified in the brain that influences the growth and differentiation of NEURONS and NEUROGLIA. Glia maturation factor beta is the 17-kDa polypeptide product of the GMFB gene and is the principal component of GLIA MATURATION FACTOR.
Injuries to the PERIPHERAL NERVES.
Diseases characterized by loss or dysfunction of myelin in the central or peripheral nervous system.
A protein found most abundantly in the nervous system. Defects or deficiencies in this protein are associated with NEUROFIBROMATOSIS 1, Watson syndrome, and LEOPARD syndrome. Mutations in the gene (GENE, NEUROFIBROMATOSIS 1) affect two known functions: regulation of ras-GTPase and tumor suppression.
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)
Diseases of the peripheral nerves external to the brain and spinal cord, which includes diseases of the nerve roots, ganglia, plexi, autonomic nerves, sensory nerves, and motor nerves.
A family of highly acidic calcium-binding proteins found in large concentration in the brain and believed to be glial in origin. They are also found in other organs in the body. They have in common the EF-hand motif (EF HAND MOTIFS) found on a number of calcium binding proteins. The name of this family derives from the property of being soluble in a 100% saturated ammonium sulfate solution.
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.
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.
Refers to animals in the period of time just after birth.
Mice which carry mutant genes for neurologic defects or abnormalities.
Tumor suppressor genes located on the long arm of human chromosome 17 in the region 17q11.2. Mutation of these genes is thought to cause NEUROFIBROMATOSIS 1, Watson syndrome, and LEOPARD syndrome.
Large, noncollagenous glycoprotein with antigenic properties. It is localized in the basement membrane lamina lucida and functions to bind epithelial cells to the basement membrane. Evidence suggests that the protein plays a role in tumor invasion.
The outermost cytoplasmic layer of the SCHWANN CELLS covering NERVE FIBERS.
Cell surface receptors that bind NERVE GROWTH FACTOR; (NGF) and a NGF-related family of neurotrophic factors that includes neurotrophins, BRAIN-DERIVED NEUROTROPHIC FACTOR and CILIARY NEUROTROPHIC FACTOR.
Paired bundles of NERVE FIBERS entering and leaving the SPINAL CORD at each segment. The dorsal and ventral nerve roots join to form the mixed segmental spinal nerves. The dorsal roots are generally afferent, formed by the central projections of the spinal (dorsal root) ganglia sensory cells, and the ventral roots are efferent, comprising the axons of spinal motor and PREGANGLIONIC AUTONOMIC FIBERS.
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.
A subclass of closely-related SOX transcription factors. Members of this subfamily have been implicated in regulating the differentiation of OLIGODENDROCYTES during neural crest formation and in CHONDROGENESIS.
A low affinity receptor that binds NERVE GROWTH FACTOR; BRAIN-DERIVED NEUROTROPHIC FACTOR; NEUROTROPHIN 3; and neurotrophin 4.
A branch of the tibial nerve which supplies sensory innervation to parts of the lower leg and foot.
An autosomal dominant inherited disorder (with a high frequency of spontaneous mutations) that features developmental changes in the nervous system, muscles, bones, and skin, most notably in tissue derived from the embryonic NEURAL CREST. Multiple hyperpigmented skin lesions and subcutaneous tumors are the hallmark of this disease. Peripheral and central nervous system neoplasms occur frequently, especially OPTIC NERVE GLIOMA and NEUROFIBROSARCOMA. NF1 is caused by mutations which inactivate the NF1 gene (GENES, NEUROFIBROMATOSIS 1) on chromosome 17q. The incidence of learning disabilities is also elevated in this condition. (From Adams et al., Principles of Neurology, 6th ed, pp1014-18) There is overlap of clinical features with NOONAN SYNDROME in a syndrome called neurofibromatosis-Noonan syndrome. Both the PTPN11 and NF1 gene products are involved in the SIGNAL TRANSDUCTION pathway of Ras (RAS PROTEINS).
A technique of culturing mixed cell types in vitro to allow their synergistic or antagonistic interactions, such as on CELL DIFFERENTIATION or APOPTOSIS. Coculture can be of different types of cells, tissues, or organs from normal or disease states.
Factors which enhance the growth potentialities of sensory and sympathetic nerve cells.
Transection or severing of an axon. This type of denervation is used often in experimental studies on neuronal physiology and neuronal death or survival, toward an understanding of nervous system disease.
'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.
A membrane protein homologous to the ERM (Ezrin-Radixin-Moesin) family of cytoskeleton-associated proteins which regulate physical properties of membranes. Alterations in neurofibromin 2 are the cause of NEUROFIBROMATOSIS 2.
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.
Differentiated tissue of the central nervous system composed of NERVE CELLS, fibers, DENDRITES, and specialized supporting cells.
A strain of albino rat used widely for experimental purposes because of its calmness and ease of handling. It was developed by the Sprague-Dawley Animal Company.
Progressive restriction of the developmental potential and increasing specialization of function that leads to the formation of specialized cells, tissues, and organs.
Histochemical localization of immunoreactive substances using labeled antibodies as reagents.
Any of several ways in which living cells of an organism communicate with one another, whether by direct contact between cells or by means of chemical signals carried by neurotransmitter substances, hormones, and cyclic AMP.
The basic cellular units of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the NERVOUS SYSTEM.
Neoplasms which arise from nerve sheaths formed by SCHWANN CELLS in the PERIPHERAL NERVOUS SYSTEM or by OLIGODENDROCYTES in the CENTRAL NERVOUS SYSTEM. Malignant peripheral nerve sheath tumors, NEUROFIBROMA, and NEURILEMMOMA are relatively common tumors in this category.
A species of gram-positive, aerobic bacteria that causes LEPROSY in man. Its organisms are generally arranged in clumps, rounded masses, or in groups of bacilli side by side.
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 two longitudinal ridges along the PRIMITIVE STREAK appearing near the end of GASTRULATION during development of nervous system (NEURULATION). The ridges are formed by folding of NEURAL PLATE. Between the ridges is a neural groove which deepens as the fold become elevated. When the folds meet at midline, the groove becomes a closed tube, the NEURAL TUBE.
Slender processes of NEURONS, including the AXONS and their glial envelopes (MYELIN SHEATH). Nerve fibers conduct nerve impulses to and from the CENTRAL NERVOUS SYSTEM.
Type III intermediate filament proteins that assemble into neurofilaments, the major cytoskeletal element in nerve axons and dendrites. They consist of three distinct polypeptides, the neurofilament triplet. Types I, II, and IV intermediate filament proteins form other cytoskeletal elements such as keratins and lamins. It appears that the metabolism of neurofilaments is disturbed in Alzheimer's disease, as indicated by the presence of neurofilament epitopes in the neurofibrillary tangles, as well as by the severe reduction of the expression of the gene for the light neurofilament subunit of the neurofilament triplet in brains of Alzheimer's patients. (Can J Neurol Sci 1990 Aug;17(3):302)
A nervous tissue specific protein which is highly expressed in NEURONS during development and NERVE REGENERATION. It has been implicated in neurite outgrowth, long-term potentiation, SIGNAL TRANSDUCTION, and NEUROTRANSMITTER release. (From Neurotoxicology 1994;15(1):41-7) It is also a substrate of PROTEIN KINASE C.
Laboratory mice that have been produced from a genetically manipulated EGG or EMBRYO, MAMMALIAN.
The fission of a CELL. It includes CYTOKINESIS, when the CYTOPLASM of a cell is divided, and CELL NUCLEUS DIVISION.
Transference of cells within an individual, between individuals of the same species, or between individuals of different species.
A nerve originating in the lumbar spinal cord (usually L2 to L4) and traveling through the lumbar plexus to provide motor innervation to extensors of the thigh and sensory innervation to parts of the thigh, lower leg, and foot, and to the hip and knee joints.
A cylindrical column of tissue that lies within the vertebral canal. It is composed of WHITE MATTER and GRAY MATTER.
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.
GLYCOSPHINGOLIPIDS with a sulfate group esterified to one of the sugar groups.
A superorder of CEPHALOPODS comprised of squid, cuttlefish, and their relatives. Their distinguishing feature is the modification of their fourth pair of arms into tentacles, resulting in 10 limbs.
A cell surface protein-tyrosine kinase receptor that is specific for NEUREGULINS. It has extensive homology to and can heterodimerize with the EGF RECEPTOR and the ERBB-2 RECEPTOR. Overexpression of the erbB-3 receptor is associated with TUMORIGENESIS.
The synapse between a neuron and a muscle.
Surface ligands that mediate cell-to-cell adhesion and function in the assembly and interconnection of the vertebrate nervous system. These molecules promote cell adhesion via a homophilic mechanism. These are not to be confused with NEURAL CELL ADHESION MOLECULES, now known to be expressed in a variety of tissues and cell types in addition to nervous tissue.
The resection or removal of the nerve to an organ or part. (Dorland, 28th ed)
The movement of cells from one location to another. Distinguish from CYTOKINESIS which is the process of dividing the CYTOPLASM of a cell.

Confocal calcium imaging reveals an ionotropic P2 nucleotide receptor in the paranodal membrane of rat Schwann cells. (1/1583)

1. The paranodal Schwann cell region is of major importance for the function of a myelinated axon. In the present study we searched for a possible ionotropic effect of extracellular ATP in this Schwann cell compartment. 2. Whole-cell patch-clamp recordings from cultured rat Schwann cells revealed that ATP and 2'-3'-O-(4-benzoylbenzoyl)-adenosine 5'-triphosphate (BzATP) induced a non-specific cation current. The effect of ATP was much enhanced in a Ca2+- and Mg2+-free solution. ADP, UTP and alpha,beta-methylene adenosine 5'-triphosphate (alpha,beta-meATP) had no effect. 3. Confocal Ca2+ imaging of myelinating Schwann cells in isolated rat spinal roots showed a BzATP-induced rise in the free intracellular Ca2+ concentration in the paranodal Schwann cell cytoplasm whereas alpha,beta-meATP and 2-(methylthio)-adenosine 5'-triphosphate were without effect. In contrast to the known metabotropic effect of UTP on these Schwann cell regions, the BzATP-induced Ca2+ signal was not transient, was unaffected by depletion of intracellular Ca2+ stores and dependent on the presence of extracellular Ca2+. 4. These results suggest that an ionotropic ATP receptor with electrophysiological and pharmacological characteristics of the P2X7 subtype of nucleotide receptors is functionally active in myelinating Schwann cells of peripheral nerves. Such a receptor might contribute to Schwann cell reactions in nerve injury or neuropathy.  (+info)

Transport of Trembler-J mutant peripheral myelin protein 22 is blocked in the intermediate compartment and affects the transport of the wild-type protein by direct interaction. (2/1583)

Peripheral myelin protein 22 (PMP22) is an integral membrane protein that is essential for the normal formation and maintenance of peripheral myelin. Duplications, deletions, or mutations in the PMP22 gene account for a set of dominantly inherited peripheral neuropathies. The heterozygous Trembler-J (TrJ) genotype in mice is similar genetically to a Charcot-Marie-Tooth disease type 1A pedigree in humans, whereas the homozygous TrJ condition leads to the most severe form of PMP22-associated neuropathies. To characterize the consequences of the TrJ mutation, we labeled wild-type (wt-) and TrJ-PMP22 in the third loop of the protein with different epitope tags and expressed them separately or together in COS7 cells and primary Schwann cells. Here we show that the transport of the mutant TrJ-PMP22 is interrupted in the intermediate compartment, preventing its insertion into the plasma membrane and affecting the morphology of the endoplasmic reticulum. In addition, TrJ-PMP22 forms a heterodimer with the wt-PMP22. This interaction causes a fraction of the wt-PMP22 to be retained with TrJ-PMP22 in the intermediate compartment of COS7 and Schwann cells. The relative stability of a wt-mutant PMP22 heterodimer as compared with the wt-wt PMP22 homodimer may determine whether a particular mutation is semidominant or dominant. The neuropathy itself appears to result both from decreased trafficking of wt-PMP22 to the plasma membrane and from a toxic gain of function via the accumulation of wt- and TrJ-PMP22 in the intermediate compartment.  (+info)

A role for insulin-like growth factor-I in the regulation of Schwann cell survival. (3/1583)

During postnatal development in the peripheral nerve, differentiating Schwann cells are susceptible to apoptotic death. Schwann cell apoptosis is regulated by axons and serves as one mechanism through which axon and Schwann cell numbers are correctly matched. This regulation is mediated in part by the provision of limiting axon-derived trophic molecules, although neuregulin-1 (NRG-1) is the only trophic factor shown to date to support Schwann cell survival. In this report, we identify insulin-like growth factor-I (IGF-I) as an additional trophin that can promote Schwann cell survival in vitro. We find that IGF-I, like NRG-1, can prevent the apoptotic death of postnatal rat Schwann cells cultured under conditions of serum withdrawal. Moreover, we show that differentiating Schwann cells in the rat sciatic nerve express both the IGF-I receptor (IGF-I R) and IGF-I throughout postnatal development. These results indicate that IGF-I is likely to control Schwann cell viability in the developing peripheral nerve and, together with other findings, raise the interesting possibility that such survival regulation may switch during postnatal development from an axon-dependent mechanism to an autocrine and/or paracrine one.  (+info)

A glial cell line-derived neurotrophic factor-secreting clone of the Schwann cell line SCTM41 enhances survival and fiber outgrowth from embryonic nigral neurons grafted to the striatum and to the lesioned substantia nigra. (4/1583)

We have developed a novel Schwann cell line, SCTM41, derived from postnatal sciatic nerve cultures and have stably transfected a clone with a rat glial cell line-derived neurotrophic factor (GDNF) construct. Coculture with this GDNF-secreting clone enhances in vitro survival and fiber growth of embryonic dopaminergic neurons. In the rat unilateral 6-OHDA lesion model of Parkinson's disease, we have therefore made cografts of these cells with embryonic day 14 ventral mesencephalic grafts and assayed for effects on dopaminergic cell survival and process outgrowth. We show that cografts of GDNF-secreting Schwann cell lines improve the survival of intrastriatal embryonic dopaminergic neuronal grafts and improve neurite outgrowth into the host neuropil but have no additional effect on amphetamine-induced rotation. We next looked to see whether bridge grafts of GDNF-secreting SCTM41 cells would promote the growth of axons to their striatal targets from dopaminergic neurons implanted orthotopically into the 6-OHDA-lesioned substantia nigra. We show that such bridge grafts increase the survival of implanted embryonic dopaminergic neurons and promote the growth of axons through the grafts to the striatum.  (+info)

Krox-20 controls SCIP expression, cell cycle exit and susceptibility to apoptosis in developing myelinating Schwann cells. (5/1583)

The transcription factors Krox-20 and SCIP each play important roles in the differentiation of Schwann cells. However, the genes encoding these two proteins exhibit distinct time courses of expression and yield distinct cellular phenotypes upon mutation. SCIP is expressed prior to the initial appearance of Krox-20, and is transient in both the myelinating and non-myelinating Schwann cell lineages; while in contrast, Krox-20 appears approximately 24 hours after SCIP and then only within the myelinating lineage, where its expression is stably maintained into adulthood. Similarly, differentiation of SCIP-/- Schwann cells appears to transiently stall at the promyelinating stage that precedes myelination, whereas Krox-20(-/-) cells are, by morphological criteria, arrested at this stage. These observations led us to examine SCIP regulation and Schwann cell phenotype in Krox-20 mouse mutants. We find that in Krox-20(-/-) Schwann cells, SCIP expression is converted from transient to sustained. We further observe that both Schwann cell proliferation and apoptosis, which are normal features of SCIP+ cells, are also markedly increased late in postnatal development in Krox-20 mutants relative to wild type, and that the levels of cell division and apoptosis are balanced to yield a stable number of Schwann cells within peripheral nerves. These data demonstrate that the loss of Krox-20 in myelinating Schwann cells arrests differentiation at the promyelinating stage, as assessed by SCIP expression, mitotic activity and susceptibility to apoptosis.  (+info)

Characterization of the transmembrane molecular architecture of the dystroglycan complex in schwann cells. (6/1583)

We have demonstrated previously 1) that the dystroglycan complex, but not the sarcoglycan complex, is expressed in peripheral nerve, and 2) that alpha-dystroglycan is an extracellular laminin-2-binding protein anchored to beta-dystroglycan in the Schwann cell membrane. In the present study, we investigated the transmembrane molecular architecture of the dystroglycan complex in Schwann cells. The cytoplasmic domain of beta-dystroglycan was co-localized with Dp116, the Schwann cell-specific isoform of dystrophin, in the abaxonal Schwann cell cytoplasm adjacent to the outer membrane. beta-dystroglycan bound to Dp116 mainly via the 15 C-terminal amino acids of its cytoplasmic domain, but these amino acids were not solely responsible for the interaction of these two proteins. Interestingly, the beta-dystroglycan-precipitating antibody precipitated only a small fraction of alpha-dystroglycan and did not precipitate laminin and Dp116 from the peripheral nerve extracts. Our results indicate 1) that Dp116 is a component of the submembranous cytoskeletal system that anchors the dystroglycan complex in Schwann cells, and 2) that the dystroglycan complex in Schwann cells is fragile compared with that in striated muscle cells. We propose that this fragility may be attributable to the absence of the sarcoglycan complex in Schwann cells.  (+info)

Neural cell surface differentiation antigen gp130(RB13-6) induces fibroblasts and glioma cells to express astroglial proteins and invasive properties. (7/1583)

Transient expression of the differentiation and tumor cell surface antigen gp130(RB13-6) characterizes a subset of rat glial progenitor cells susceptible to ethylnitrosourea-induced neurooncogenesis. gp130(RB13-6) is as a member of an emerging protein family of ecto-phosphodiesterases/nucleotide pyrophosphatases that includes PC-1 and the tumor cell motility factor autotaxin. We have investigated the potential role of gp130(RB13-6) in glial differentiation by transfection of three cell lines of different origin that do not express endogenous gp130(RB13-6) (NIH-3T3 mouse fibroblasts; C6 and BT7Ca rat glioma cells) with the cDNA encoding gp130(RB13-6). The effect of gp130(RB13-6) expression was analyzed in terms of overall cell morphology, the expression of glial cell-specific marker proteins, and invasiveness. Transfectant sublines, consisting of 100% gp130(RB13-6)-positive cells, exhibited an altered, bipolar morphology. Fascicular aggregates of fibroblastoid cells subsequently developed into mesh-like patterns. Contrary to the parental NIH-3T3 and BT7Ca cells, the transfectant cells invaded into collagen type I. As shown by immunofluorescence staining of the transfectant sublines as well as of primary cultures composed of gp130(RB13-6)-positive and -negative cells, expression of gp130(RB13-6) induced coexpression of proteins typical for glial cells and their precursors, i.e., glial fibrillary acidic protein, the low affinity nerve growth factor receptor, and the neural proteins Thy-1, Ran-2, and S-100. In accordance with its expression in the immature rat nervous system, gp130(RB13-6) may thus have a significant role in the glial differentiation program and its subversion in neurooncogenesis.  (+info)

Schwann cell hyperplasia and tumors in transgenic mice expressing a naturally occurring mutant NF2 protein. (8/1583)

Specific mutations in some tumor suppressor genes such as p53 can act in a dominant fashion. We tested whether this mechanism may also apply for the neurofibromatosis type-2 gene (NF2) which, when mutated, leads to schwannoma development. Transgenic mice were generated that express, in Schwann cells, mutant NF2 proteins prototypic of natural mutants observed in humans. Mice expressing a NF2 protein with an interstitial deletion in the amino-terminal domain showed high prevalence of Schwann cell-derived tumors and Schwann cell hyperplasia, whereas those expressing a carboxy-terminally truncated protein were normal. Our results indicate that a subset of mutant NF2 alleles observed in patients may encode products with dominant properties when overexpressed in specific cell lineages.  (+info)

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.

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 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.

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.

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.

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.

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.

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.

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.

A neurofibroma is a benign (non-cancerous) tumor that develops from the nerve sheath, which is the protective covering around nerves. These tumors can grow anywhere on the body and can be found under the skin or deep inside the body. Neurofibromas can vary in size, and they may cause symptoms such as pain, numbness, or tingling if they press on nearby nerves.

Neurofibromas are a common feature of neurofibromatosis type 1 (NF1), a genetic disorder that affects approximately 1 in every 3,000 people worldwide. NF1 is characterized by the development of multiple neurofibromas and other tumors, as well as skin changes such as café-au-lait spots and freckling.

It's important to note that while most neurofibromas are benign, they can rarely undergo malignant transformation and become cancerous. If you have a neurofibroma or are concerned about your risk of developing one, it's important to seek medical advice from a healthcare professional who is familiar with this condition.

Octamer Transcription Factor-6 (OTF-6) is not a commonly used or widely accepted medical term. However, in the field of molecular biology, an octamer transcription factor refers to a protein that binds to specific octamer motifs in DNA and regulates gene transcription. The "6" likely refers to the specific isoform or variant of this transcription factor.

More specifically, OTF-6 may refer to the protein product of the SOX6 gene, which encodes a member of the SOX (SRY-related HMG box) family of transcription factors. These proteins contain a high mobility group (HMG) box DNA-binding domain and play critical roles in various developmental processes, including cell fate specification, organogenesis, and tumorigenesis.

The SOX6 protein can form homodimers or heterodimers with other SOX family members to bind to specific octamer motifs (consensus sequence: AACAAAG) in the regulatory regions of target genes. By modulating the expression of these target genes, OTF-6/SOX6 helps regulate various cellular processes, such as neurogenesis, chondrogenesis, and myogenesis.

It is essential to note that the term "Octamer Transcription Factor-6" may not be universally recognized or consistently used in scientific literature, so it is always best to refer to primary sources for precise definitions and contexts.

Neuregulin-1 (NRG-1) is a growth factor that belongs to the neuregulin family and is involved in the development and function of the nervous system. It is a protein that is encoded by the NRG1 gene and is expressed in various tissues, including the brain. NRG-1 plays important roles in the regulation of neuronal survival, migration, differentiation, and synaptic plasticity. It acts as a ligand for the ErbB family of receptor tyrosine kinases, which are involved in intracellular signaling pathways that control various cellular processes. Abnormalities in NRG-1 signaling have been implicated in several neurological and psychiatric disorders, including schizophrenia, bipolar disorder, and Alzheimer's disease.

Spinal ganglia, also known as dorsal root ganglia, are clusters of nerve cell bodies located in the peripheral nervous system. They are situated along the length of the spinal cord and are responsible for transmitting sensory information from the body to the brain. Each spinal ganglion contains numerous neurons, or nerve cells, with long processes called axons that extend into the periphery and innervate various tissues and organs. The cell bodies within the spinal ganglia receive sensory input from these axons and transmit this information to the central nervous system via the dorsal roots of the spinal nerves. This allows the brain to interpret and respond to a wide range of sensory stimuli, including touch, temperature, pain, and proprioception (the sense of the position and movement of one's body).

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.

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.

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.

A neurilemmoma, also known as schwannoma or peripheral nerve sheath tumor, is a benign, slow-growing tumor that arises from the Schwann cells, which produce the myelin sheath that surrounds and insulates peripheral nerves. These tumors can occur anywhere along the course of a peripheral nerve, but they most commonly affect the acoustic nerve (vestibulocochlear nerve), leading to a type of tumor called vestibular schwannoma or acoustic neuroma. Neurilemmomas are typically encapsulated and do not invade the surrounding tissue, although larger ones may cause pressure-related symptoms due to compression of nearby structures. Rarely, these tumors can undergo malignant transformation, leading to a condition called malignant peripheral nerve sheath tumor or neurofibrosarcoma.

Neuregulins are a family of growth factors that play important roles in the development and maintenance of the nervous system. They bind to and activate receptors known as ErbB receptors, which are tyrosine kinase receptors. Neuregulins are involved in the regulation of various cellular processes, including proliferation, differentiation, migration, and survival.

There are several different forms of neuregulins, which are produced by alternative splicing of a single gene. These forms include heregulin, glial growth factor, and neu differentiation factor. Neuregulins are produced by various cell types in the nervous system, including neurons and glial cells. They are involved in the development and maintenance of the nervous system, including the formation of synapses, the regulation of myelination, and the survival of neurons.

Dysregulation of neuregulin signaling has been implicated in various neurological disorders, including schizophrenia, Alzheimer's disease, and epilepsy.

Sciatic neuropathy is a condition that results from damage or injury to the sciatic nerve, which is the largest nerve in the human body. The sciatic nerve originates from the lower spine (lumbar and sacral regions) and travels down through the buttocks, hips, and legs to the feet.

Sciatic neuropathy can cause various symptoms, including pain, numbness, tingling, weakness, or difficulty moving the affected leg or foot. The pain associated with sciatic neuropathy is often described as sharp, shooting, or burning and may worsen with movement, coughing, or sneezing.

The causes of sciatic neuropathy include compression or irritation of the nerve due to conditions such as herniated discs, spinal stenosis, bone spurs, tumors, or piriformis syndrome. Trauma or injury to the lower back, hip, or buttocks can also cause sciatic neuropathy.

Diagnosing sciatic neuropathy typically involves a physical examination and medical history, as well as imaging tests such as X-rays, MRI, or CT scans to visualize the spine and surrounding structures. Treatment options may include pain management, physical therapy, steroid injections, or surgery, depending on the severity and underlying cause of the condition.

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.

"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.

A nerve crush injury is a type of peripheral nerve injury that occurs when there is excessive pressure or compression applied to a nerve, causing it to become damaged or dysfunctional. This can happen due to various reasons such as trauma from accidents, surgical errors, or prolonged pressure on the nerve from tight casts, clothing, or positions.

The compression disrupts the normal functioning of the nerve, leading to symptoms such as numbness, tingling, weakness, or pain in the affected area. In severe cases, a nerve crush injury can cause permanent damage to the nerve, leading to long-term disability or loss of function. Treatment for nerve crush injuries typically involves relieving the pressure on the nerve, providing supportive care, and in some cases, surgical intervention may be necessary to repair the damaged nerve.

Glia maturation factor (GMF) is a protein that belongs to the family of non-catalytic leucine-rich repeat and immunoglobulin-like domain-containing Nogo receptor-interacting proteins (NLRs). GMF is primarily expressed in glial cells in the central nervous system. It plays a crucial role in regulating cytoskeletal dynamics, particularly actin polymerization and depolymerization, which are essential for various cellular processes such as cell motility, division, and differentiation.

GMF has been shown to interact with the actin-depolymerizing factor cofilin and regulate its activity by controlling its phosphorylation state. Specifically, GMF inhibits cofilin's ability to sever and depolymerize actin filaments, thereby promoting actin polymerization and stabilization of the cytoskeleton.

In addition to its role in cytoskeletal regulation, GMF has been implicated in various neurological disorders, including Alzheimer's disease, Parkinson's disease, and spinal cord injury. However, further research is needed to fully understand the molecular mechanisms underlying these associations.

Peripheral nerve injuries refer to damage or trauma to the peripheral nerves, which are the nerves outside the brain and spinal cord. These nerves transmit information between the central nervous system (CNS) and the rest of the body, including sensory, motor, and autonomic functions. Peripheral nerve injuries can result in various symptoms, depending on the type and severity of the injury, such as numbness, tingling, weakness, or paralysis in the affected area.

Peripheral nerve injuries are classified into three main categories based on the degree of damage:

1. Neuropraxia: This is the mildest form of nerve injury, where the nerve remains intact but its function is disrupted due to a local conduction block. The nerve fiber is damaged, but the supporting structures remain intact. Recovery usually occurs within 6-12 weeks without any residual deficits.
2. Axonotmesis: In this type of injury, there is damage to both the axons and the supporting structures (endoneurium, perineurium). The nerve fibers are disrupted, but the connective tissue sheaths remain intact. Recovery can take several months or even up to a year, and it may be incomplete, with some residual deficits possible.
3. Neurotmesis: This is the most severe form of nerve injury, where there is complete disruption of the nerve fibers and supporting structures (endoneurium, perineurium, epineurium). Recovery is unlikely without surgical intervention, which may involve nerve grafting or repair.

Peripheral nerve injuries can be caused by various factors, including trauma, compression, stretching, lacerations, or chemical exposure. Treatment options depend on the type and severity of the injury and may include conservative management, such as physical therapy and pain management, or surgical intervention for more severe cases.

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.

Neurofibromin 1 is a protein that is encoded by the NF1 gene in humans. Neurofibromin 1 acts as a tumor suppressor, helping to regulate cell growth and division. It plays an important role in the nervous system, where it helps to control the development and function of nerve cells. Mutations in the NF1 gene can lead to neurofibromatosis type 1 (NF1), a genetic disorder characterized by the growth of non-cancerous tumors on the nerves (neurofibromas) and other symptoms.

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.

Peripheral Nervous System (PNS) diseases, also known as Peripheral Neuropathies, refer to conditions that affect the functioning of the peripheral nervous system, which includes all the nerves outside the brain and spinal cord. These nerves transmit signals between the central nervous system (CNS) and the rest of the body, controlling sensations, movements, and automatic functions such as heart rate and digestion.

PNS diseases can be caused by various factors, including genetics, infections, toxins, metabolic disorders, trauma, or autoimmune conditions. The symptoms of PNS diseases depend on the type and extent of nerve damage but often include:

1. Numbness, tingling, or pain in the hands and feet
2. Muscle weakness or cramps
3. Loss of reflexes
4. Decreased sensation to touch, temperature, or vibration
5. Coordination problems and difficulty with balance
6. Sexual dysfunction
7. Digestive issues, such as constipation or diarrhea
8. Dizziness or fainting due to changes in blood pressure

Examples of PNS diseases include Guillain-Barre syndrome, Charcot-Marie-Tooth disease, diabetic neuropathy, and peripheral nerve injuries. Treatment for these conditions varies depending on the underlying cause but may involve medications, physical therapy, lifestyle changes, or surgery.

S100 proteins are a family of calcium-binding proteins that are involved in the regulation of various cellular processes, including cell growth and differentiation, intracellular signaling, and inflammation. They are found in high concentrations in certain types of cells, such as nerve cells (neurons), glial cells (supporting cells in the nervous system), and skin cells (keratinocytes).

The S100 protein family consists of more than 20 members, which are divided into several subfamilies based on their structural similarities. Some of the well-known members of this family include S100A1, S100B, S100 calcium-binding protein A8 (S100A8), and S100 calcium-binding protein A9 (S100A9).

Abnormal expression or regulation of S100 proteins has been implicated in various pathological conditions, such as neurodegenerative diseases, cancer, and inflammatory disorders. For example, increased levels of S100B have been found in the brains of patients with Alzheimer's disease, while overexpression of S100A8 and S100A9 has been associated with the development and progression of certain types of cancer.

Therefore, understanding the functions and regulation of S100 proteins is important for developing new diagnostic and therapeutic strategies for various diseases.

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 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.

"Newborn animals" refers to the very young offspring of animals that have recently been born. In medical terminology, newborns are often referred to as "neonates," and they are classified as such from birth until about 28 days of age. During this time period, newborn animals are particularly vulnerable and require close monitoring and care to ensure their survival and healthy development.

The specific needs of newborn animals can vary widely depending on the species, but generally, they require warmth, nutrition, hydration, and protection from harm. In many cases, newborns are unable to regulate their own body temperature or feed themselves, so they rely heavily on their mothers for care and support.

In medical settings, newborn animals may be examined and treated by veterinarians to ensure that they are healthy and receiving the care they need. This can include providing medical interventions such as feeding tubes, antibiotics, or other treatments as needed to address any health issues that arise. Overall, the care and support of newborn animals is an important aspect of animal medicine and conservation efforts.

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.

Neurofibromatosis 1 (NF1) is a genetic disorder caused by mutations in the NF1 gene, which is located on chromosome 17 and encodes the protein neurofibromin. Neurofibromin is a tumor suppressor protein that regulates cell growth and differentiation.

The NF1 gene mutation leads to the development of benign (non-cancerous) tumors on nerves and skin, called neurofibromas, as well as other clinical features such as café-au-lait spots (light brown patches on the skin), freckling in the axillary or inguinal regions, Lisch nodules (harmless growths on the iris of the eye), and skeletal abnormalities.

Neurofibromatosis 1 is an autosomal dominant disorder, which means that a person has a 50% chance of inheriting the mutated gene from an affected parent. However, up to 50% of cases result from new mutations in the NF1 gene and occur in people with no family history of the condition.

The clinical manifestations of Neurofibromatosis 1 can vary widely among individuals, even within the same family. The diagnosis is typically made based on clinical criteria established by the National Institutes of Health (NIH). Treatment is generally focused on managing symptoms and addressing complications as they arise, although surgery may be necessary to remove large or symptomatic tumors.

Laminin is a family of proteins that are an essential component of the basement membrane, which is a specialized type of extracellular matrix. Laminins are large trimeric molecules composed of three different chains: α, β, and γ. There are five different α chains, three different β chains, and three different γ chains that can combine to form at least 15 different laminin isoforms.

Laminins play a crucial role in maintaining the structure and integrity of basement membranes by interacting with other components of the extracellular matrix, such as collagen IV, and cell surface receptors, such as integrins. They are involved in various biological processes, including cell adhesion, differentiation, migration, and survival.

Laminin dysfunction has been implicated in several human diseases, including cancer, diabetic nephropathy, and muscular dystrophy.

A neurilemma, also known as a schwannoma or neurolemmoma, is a type of benign tumor that arises from the nerve sheath. Specifically, it develops from the Schwann cells, which produce the myelin sheath that insulates and protects the nerves. Neurilemmomas can occur anywhere in the body where there are nerves, but they most commonly affect the cranial nerves, particularly the eighth cranial nerve (the vestibulocochlear nerve). They can also be found along the spine and in the extremities.

Neurilemmomas typically appear as solitary, slow-growing, and well-circumscribed masses that do not usually cause pain or other symptoms unless they compress nearby structures. In some cases, however, they may cause hearing loss, tinnitus, balance problems, or facial nerve paralysis when they affect the cranial nerves. Treatment typically involves surgical removal of the tumor, and the prognosis is generally good, with a low risk of recurrence.

Nerve Growth Factor (NGF) receptors are a type of protein molecule found on the surface of certain cells, specifically those associated with the nervous system. They play a crucial role in the development, maintenance, and survival of neurons (nerve cells). There are two main types of NGF receptors:

1. Tyrosine Kinase Receptor A (TrkA): This is a high-affinity receptor for NGF and is primarily found on sensory neurons and sympathetic neurons. TrkA activation by NGF leads to the initiation of various intracellular signaling pathways that promote neuronal survival, differentiation, and growth.
2. P75 Neurotrophin Receptor (p75NTR): This is a low-affinity receptor for NGF and other neurotrophins. It can function as a coreceptor with Trk receptors to modulate their signals or act independently to mediate cell death under certain conditions.

Together, these two types of NGF receptors help regulate the complex interactions between neurons and their targets during development and throughout adult life.

Spinal nerve roots are the initial parts of spinal nerves that emerge from the spinal cord through the intervertebral foramen, which are small openings between each vertebra in the spine. These nerve roots carry motor, sensory, and autonomic fibers to and from specific regions of the body. There are 31 pairs of spinal nerve roots in total, with 8 cervical, 12 thoracic, 5 lumbar, 5 sacral, and 1 coccygeal pair. Each root has a dorsal (posterior) and ventral (anterior) ramus that branch off to form the peripheral nervous system. Irritation or compression of these nerve roots can result in pain, numbness, weakness, or loss of reflexes in the affected area.

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.

SOXE transcription factors are a subgroup of the SOX (SRY-related HMG box) family of proteins, which are involved in various developmental processes, including cell fate specification and differentiation. The SOXE group includes SOX8, SOX9, and SOX10, all of which contain a conserved high mobility group (HMG) box DNA-binding domain. They play crucial roles in the development of several tissues, such as the nervous system, skeletal system, and urogenital system.

SOXE transcription factors are known to regulate gene expression by binding to specific DNA sequences, often acting in combination with other transcription factors to control various cellular processes. Dysregulation of SOXE transcription factors has been implicated in several human diseases, including cancer and neurodevelopmental disorders.

A nerve growth factor (NGF) receptor is a type of protein found on the surface of certain cells that selectively binds to NGF, a neurotrophin or a small signaling protein that promotes the growth and survival of nerve cells. There are two main types of NGF receptors: tyrosine kinase receptor A (TrkA) and p75 neurotrophin receptor (p75NTR). TrkA is a high-affinity receptor that activates various signaling pathways leading to the survival, differentiation, and growth of nerve cells. In contrast, p75NTR has lower affinity for NGF and can either promote or inhibit NGF signaling depending on its interactions with other proteins. Together, these two types of receptors help regulate the development, maintenance, and function of the nervous system.

The sural nerve is a purely sensory peripheral nerve in the lower leg and foot. It provides sensation to the outer ( lateral) aspect of the little toe and the adjacent side of the fourth toe, as well as a small portion of the skin on the back of the leg between the ankle and knee joints.

The sural nerve is formed by the union of branches from the tibial and common fibular nerves (branches of the sciatic nerve) in the lower leg. It runs down the calf, behind the lateral malleolus (the bony prominence on the outside of the ankle), and into the foot.

The sural nerve is often used as a donor nerve during nerve grafting procedures due to its consistent anatomy and relatively low risk for morbidity at the donor site.

Neurofibromatosis 1 (NF1) is a genetic disorder that affects the development and growth of nerve tissue. It's also known as von Recklinghausen disease. NF1 is characterized by the growth of non-cancerous tumors on the nerves, as well as skin and bone abnormalities.

The symptoms of Neurofibromatosis 1 can vary widely, even among members of the same family. Some common features include:

* Multiple café au lait spots (flat, light brown patches on the skin)
* Freckles in the underarms and groin area
* Benign growths on or under the skin called neurofibromas
* Larger, more complex tumors called plexiform neurofibromas
* Optic gliomas (tumors that form on the optic nerve)
* Distinctive bone abnormalities, such as a curved spine (scoliosis) or an enlarged head (macrocephaly)
* Learning disabilities and behavioral problems

Neurofibromatosis 1 is caused by mutations in the NF1 gene, which provides instructions for making a protein called neurofibromin. This protein helps regulate cell growth and division. When the NF1 gene is mutated, the production of neurofibromin is reduced or absent, leading to uncontrolled cell growth and the development of tumors.

NF1 is an autosomal dominant disorder, which means that a person has a 50% chance of inheriting the mutated gene from an affected parent. However, about half of all cases are the result of new mutations in the NF1 gene, and occur in people with no family history of the disorder.

There is currently no cure for Neurofibromatosis 1, but treatments are available to manage the symptoms and complications of the disease. These may include medications to control pain or reduce the size of tumors, surgery to remove tumors or correct bone abnormalities, and physical therapy to improve mobility and strength. Regular monitoring by a healthcare team experienced in treating Neurofibromatosis 1 is also important to detect any changes in the condition and provide appropriate care.

Coculture techniques refer to a type of experimental setup in which two or more different types of cells or organisms are grown and studied together in a shared culture medium. This method allows researchers to examine the interactions between different cell types or species under controlled conditions, and to study how these interactions may influence various biological processes such as growth, gene expression, metabolism, and signal transduction.

Coculture techniques can be used to investigate a wide range of biological phenomena, including the effects of host-microbe interactions on human health and disease, the impact of different cell types on tissue development and homeostasis, and the role of microbial communities in shaping ecosystems. These techniques can also be used to test the efficacy and safety of new drugs or therapies by examining their effects on cells grown in coculture with other relevant cell types.

There are several different ways to establish cocultures, depending on the specific research question and experimental goals. Some common methods include:

1. Mixed cultures: In this approach, two or more cell types are simply mixed together in a culture dish or flask and allowed to grow and interact freely.
2. Cell-layer cultures: Here, one cell type is grown on a porous membrane or other support structure, while the second cell type is grown on top of it, forming a layered coculture.
3. Conditioned media cultures: In this case, one cell type is grown to confluence and its culture medium is collected and then used to grow a second cell type. This allows the second cell type to be exposed to any factors secreted by the first cell type into the medium.
4. Microfluidic cocultures: These involve growing cells in microfabricated channels or chambers, which allow for precise control over the spatial arrangement and flow of nutrients, waste products, and signaling molecules between different cell types.

Overall, coculture techniques provide a powerful tool for studying complex biological systems and gaining insights into the mechanisms that underlie various physiological and pathological processes.

Nerve Growth Factors (NGFs) are a family of proteins that play an essential role in the growth, maintenance, and survival of certain neurons (nerve cells). They were first discovered by Rita Levi-Montalcini and Stanley Cohen in 1956. NGF is particularly crucial for the development and function of the peripheral nervous system, which connects the central nervous system to various organs and tissues throughout the body.

NGF supports the differentiation and survival of sympathetic and sensory neurons during embryonic development. In adults, NGF continues to regulate the maintenance and repair of these neurons, contributing to neuroplasticity – the brain's ability to adapt and change over time. Additionally, NGF has been implicated in pain transmission and modulation, as well as inflammatory responses.

Abnormal levels or dysfunctional NGF signaling have been associated with various medical conditions, including neurodegenerative diseases (e.g., Alzheimer's and Parkinson's), chronic pain disorders, and certain cancers (e.g., small cell lung cancer). Therefore, understanding the role of NGF in physiological and pathological processes may provide valuable insights into developing novel therapeutic strategies for these conditions.

Axotomy is a medical term that refers to the surgical cutting or severing of an axon, which is the long, slender projection of a neuron (nerve cell) that conducts electrical impulses away from the cell body and toward other cells. Axons are a critical component of the nervous system, allowing for communication between different parts of the body.

Axotomy is often used in research settings to study the effects of axonal injury on neuronal function and regeneration. This procedure can provide valuable insights into the mechanisms underlying neurodegenerative disorders and potential therapies for nerve injuries. However, it is important to note that axotomy can also have significant consequences for the affected neuron, including changes in gene expression, metabolism, and overall survival.

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.

Neurofibromin 2 is not a medical term itself, but Neurofibromin 1 and Neurofibromin 2 are related to a genetic disorder called Neurofibromatosis. Neurofibromin 1 is the correct term, which is a protein encoded by the NF1 gene in humans.

Neurofibromin 1 is a tumor suppressor protein that plays a crucial role in regulating cell growth and differentiation. Mutations in the NF1 gene can lead to Neurofibromatosis type 1 (NF1), a genetic disorder characterized by the development of benign tumors on the nerves, skin, and other parts of the body.

Neurofibromin 2, on the other hand, is not a recognized term in medical literature. It is possible that there is some confusion with Neurofibromatosis type 2 (NF2), which is a separate genetic disorder caused by mutations in the NF2 gene. The NF2 gene encodes a protein called Merlin, which also functions as a tumor suppressor and helps regulate cell growth and division.

Therefore, it is essential to clarify whether you are asking about Neurofibromin 1 or Neurofibromatosis type 2 when using the term "Neurofibromin 2."

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.

Nerve tissue, also known as neural tissue, is a type of specialized tissue that is responsible for the transmission of electrical signals and the processing of information in the body. It is a key component of the nervous system, which includes the brain, spinal cord, and peripheral nerves. Nerve tissue is composed of two main types of cells: neurons and glial cells.

Neurons are the primary functional units of nerve tissue. They are specialized cells that are capable of generating and transmitting electrical signals, known as action potentials. Neurons have a unique structure, with a cell body (also called the soma) that contains the nucleus and other organelles, and processes (dendrites and axons) that extend from the cell body and are used to receive and transmit signals.

Glial cells, also known as neuroglia or glia, are non-neuronal cells that provide support and protection for neurons. There are several different types of glial cells, including astrocytes, oligodendrocytes, microglia, and Schwann cells. These cells play a variety of roles in the nervous system, such as providing structural support, maintaining the proper environment for neurons, and helping to repair and regenerate nerve tissue after injury.

Nerve tissue is found throughout the body, but it is most highly concentrated in the brain and spinal cord, which make up the central nervous system (CNS). The peripheral nerves, which are the nerves that extend from the CNS to the rest of the body, also contain nerve tissue. Nerve tissue is responsible for transmitting sensory information from the body to the brain, controlling muscle movements, and regulating various bodily functions such as heart rate, digestion, and respiration.

Sprague-Dawley rats are a strain of albino laboratory rats that are widely used in scientific research. They were first developed by researchers H.H. Sprague and R.C. Dawley in the early 20th century, and have since become one of the most commonly used rat strains in biomedical research due to their relatively large size, ease of handling, and consistent genetic background.

Sprague-Dawley rats are outbred, which means that they are genetically diverse and do not suffer from the same limitations as inbred strains, which can have reduced fertility and increased susceptibility to certain diseases. They are also characterized by their docile nature and low levels of aggression, making them easier to handle and study than some other rat strains.

These rats are used in a wide variety of research areas, including toxicology, pharmacology, nutrition, cancer, and behavioral studies. Because they are genetically diverse, Sprague-Dawley rats can be used to model a range of human diseases and conditions, making them an important tool in the development of new drugs and therapies.

Cell differentiation is the process by which a less specialized cell, or stem cell, becomes a more specialized cell type with specific functions and structures. This process involves changes in gene expression, which are regulated by various intracellular signaling pathways and transcription factors. Differentiation results in the development of distinct cell types that make up tissues and organs in multicellular organisms. It is a crucial aspect of embryonic development, tissue repair, and maintenance of homeostasis in the body.

Immunohistochemistry (IHC) is a technique used in pathology and laboratory medicine to identify specific proteins or antigens in tissue sections. It combines the principles of immunology and histology to detect the presence and location of these target molecules within cells and tissues. This technique utilizes antibodies that are specific to the protein or antigen of interest, which are then tagged with a detection system such as a chromogen or fluorophore. The stained tissue sections can be examined under a microscope, allowing for the visualization and analysis of the distribution and expression patterns of the target molecule in the context of the tissue architecture. Immunohistochemistry is widely used in diagnostic pathology to help identify various diseases, including cancer, infectious diseases, and immune-mediated disorders.

Cell communication, also known as cell signaling, is the process by which cells exchange and transmit signals between each other and their environment. This complex system allows cells to coordinate their functions and maintain tissue homeostasis. Cell communication can occur through various mechanisms including:

1. Autocrine signaling: When a cell releases a signal that binds to receptors on the same cell, leading to changes in its behavior or function.
2. Paracrine signaling: When a cell releases a signal that binds to receptors on nearby cells, influencing their behavior or function.
3. Endocrine signaling: When a cell releases a hormone into the bloodstream, which then travels to distant target cells and binds to specific receptors, triggering a response.
4. Synaptic signaling: In neurons, communication occurs through the release of neurotransmitters that cross the synapse and bind to receptors on the postsynaptic cell, transmitting electrical or chemical signals.
5. Contact-dependent signaling: When cells physically interact with each other, allowing for the direct exchange of signals and information.

Cell communication is essential for various physiological processes such as growth, development, differentiation, metabolism, immune response, and tissue repair. Dysregulation in cell communication can contribute to diseases, including cancer, diabetes, and neurological disorders.

Neurons, also known as nerve cells or neurocytes, are specialized cells that constitute the basic unit of the nervous system. They are responsible for receiving, processing, and transmitting information and signals within the body. Neurons have three main parts: the dendrites, the cell body (soma), and the axon. The dendrites receive signals from other neurons or sensory receptors, while the axon transmits these signals to other neurons, muscles, or glands. The junction between two neurons is called a synapse, where neurotransmitters are released to transmit the signal across the gap (synaptic cleft) to the next neuron. Neurons vary in size, shape, and structure depending on their function and location within the nervous system.

Nerve sheath neoplasms are a group of tumors that arise from the cells surrounding and supporting the nerves. These tumors can be benign or malignant and include schwannomas, neurofibromas, and malignant peripheral nerve sheath tumors (MPNSTs). Schwannomas develop from the Schwann cells that produce the myelin sheath of the nerve, while neurofibromas arise from the nerve's supporting cells called fibroblasts. MPNSTs are cancerous tumors that can grow rapidly and invade surrounding tissues. Nerve sheath neoplasms can cause various symptoms depending on their location and size, including pain, numbness, weakness, or paralysis in the affected area.

"Mycobacterium leprae" is a slow-growing, rod-shaped, gram-positive bacterium that is the causative agent of leprosy, a chronic infectious disease that primarily affects the skin, peripheral nerves, and mucosal surfaces of the upper respiratory tract. The bacterium was discovered in 1873 by Gerhard Armauer Hansen, a Norwegian physician, and is named after him as "Hansen's bacillus."

"Mycobacterium leprae" has a unique cell wall that contains high amounts of lipids, which makes it resistant to many common disinfectants and antibiotics. It can survive and multiply within host macrophages, allowing it to evade the immune system and establish a chronic infection.

Leprosy is a treatable disease with multidrug therapy (MDT), which combines several antibiotics such as dapsone, rifampicin, and clofazimine. Early diagnosis and treatment can prevent the progression of the disease and reduce its transmission to others.

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.

The neural crest is a transient, multipotent embryonic cell population that originates from the ectoderm (outermost layer) of the developing neural tube (precursor to the central nervous system). These cells undergo an epithelial-to-mesenchymal transition and migrate throughout the embryo, giving rise to a diverse array of cell types and structures.

Neural crest cells differentiate into various tissues, including:

1. Peripheral nervous system (PNS) components: sensory neurons, sympathetic and parasympathetic ganglia, and glial cells (e.g., Schwann cells).
2. Facial bones and cartilage, as well as connective tissue of the skull.
3. Melanocytes, which are pigment-producing cells in the skin.
4. Smooth muscle cells in major blood vessels, heart, gastrointestinal tract, and other organs.
5. Secretory cells in endocrine glands (e.g., chromaffin cells of the adrenal medulla).
6. Parts of the eye, such as the cornea and iris stroma.
7. Dental tissues, including dentin, cementum, and dental pulp.

Due to their wide-ranging contributions to various tissues and organs, neural crest cells play a crucial role in embryonic development and organogenesis. Abnormalities in neural crest cell migration or differentiation can lead to several congenital disorders, such as neurocristopathies.

Nerve fibers are specialized structures that constitute the long, slender processes (axons) of neurons (nerve cells). They are responsible for conducting electrical impulses, known as action potentials, away from the cell body and transmitting them to other neurons or effector organs such as muscles and glands. Nerve fibers are often surrounded by supportive cells called glial cells and are grouped together to form nerve bundles or nerves. These fibers can be myelinated (covered with a fatty insulating sheath called myelin) or unmyelinated, which influences the speed of impulse transmission.

Neurofilament proteins (NFs) are type IV intermediate filament proteins that are specific to neurons. They are the major structural components of the neuronal cytoskeleton and play crucial roles in maintaining the structural integrity, stability, and diameter of axons. Neurofilaments are composed of three subunits: light (NFL), medium (NFM), and heavy (NFH) neurofilament proteins, which differ in their molecular weights. These subunits assemble into heteropolymers to form the neurofilament core, while the C-terminal tails of NFH and NFM extend outward from the core, interacting with other cellular components and participating in various neuronal functions. Increased levels of neurofilament proteins, particularly NFL, in cerebrospinal fluid (CSF) and blood are considered biomarkers for axonal damage and neurodegeneration in several neurological disorders, such as Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis (ALS), and multiple sclerosis (MS).

GAP-43 protein, also known as growth-associated protein 43 or B-50, is a neuronal protein that is highly expressed during development and axonal regeneration. It is involved in the regulation of synaptic plasticity, nerve impulse transmission, and neurite outgrowth. GAP-43 is localized to the growth cones of growing axons and is thought to play a role in the guidance and navigation of axonal growth during development and regeneration. It is a member of the calcium/calmodulin-dependent protein kinase substrate family and undergoes phosphorylation by several protein kinases, including PKC (protein kinase C), which regulates its function. GAP-43 has been implicated in various neurological disorders, such as Alzheimer's disease, Parkinson's disease, and schizophrenia.

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.

Cell division is the process by which a single eukaryotic cell (a cell with a true nucleus) divides into two identical daughter cells. This complex process involves several stages, including replication of DNA, separation of chromosomes, and division of the cytoplasm. There are two main types of cell division: mitosis and meiosis.

Mitosis is the type of cell division that results in two genetically identical daughter cells. It is a fundamental process for growth, development, and tissue repair in multicellular organisms. The stages of mitosis include prophase, prometaphase, metaphase, anaphase, and telophase, followed by cytokinesis, which divides the cytoplasm.

Meiosis, on the other hand, is a type of cell division that occurs in the gonads (ovaries and testes) during the production of gametes (sex cells). Meiosis results in four genetically unique daughter cells, each with half the number of chromosomes as the parent cell. This process is essential for sexual reproduction and genetic diversity. The stages of meiosis include meiosis I and meiosis II, which are further divided into prophase, prometaphase, metaphase, anaphase, and telophase.

In summary, cell division is the process by which a single cell divides into two daughter cells, either through mitosis or meiosis. This process is critical for growth, development, tissue repair, and sexual reproduction in multicellular organisms.

Cell transplantation is the process of transferring living cells from one part of the body to another or from one individual to another. In medicine, cell transplantation is often used as a treatment for various diseases and conditions, including neurodegenerative disorders, diabetes, and certain types of cancer. The goal of cell transplantation is to replace damaged or dysfunctional cells with healthy ones, thereby restoring normal function to the affected area.

In the context of medical research, cell transplantation may involve the use of stem cells, which are immature cells that have the ability to develop into many different types of specialized cells. Stem cell transplantation has shown promise in the treatment of a variety of conditions, including spinal cord injuries, stroke, and heart disease.

It is important to note that cell transplantation carries certain risks, such as immune rejection and infection. As such, it is typically reserved for cases where other treatments have failed or are unlikely to be effective.

The femoral nerve is a major nerve in the thigh region of the human body. It originates from the lumbar plexus, specifically from the ventral rami (anterior divisions) of the second, third, and fourth lumbar nerves (L2-L4). The femoral nerve provides motor and sensory innervation to various muscles and areas in the lower limb.

Motor Innervation:
The femoral nerve is responsible for providing motor innervation to several muscles in the anterior compartment of the thigh, including:

1. Iliacus muscle
2. Psoas major muscle
3. Quadriceps femoris muscle (consisting of four heads: rectus femoris, vastus lateralis, vastus medialis, and vastus intermedius)

These muscles are involved in hip flexion, knee extension, and stabilization of the hip joint.

Sensory Innervation:
The sensory distribution of the femoral nerve includes:

1. Anterior and medial aspects of the thigh
2. Skin over the anterior aspect of the knee and lower leg (via the saphenous nerve, a branch of the femoral nerve)

The saphenous nerve provides sensation to the skin on the inner side of the leg and foot, as well as the medial malleolus (the bony bump on the inside of the ankle).

In summary, the femoral nerve is a crucial component of the lumbar plexus that controls motor functions in the anterior thigh muscles and provides sensory innervation to the anterior and medial aspects of the thigh and lower leg.

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.

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.

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.

Decapodiformes is a taxonomic order of marine cephalopods, which includes squids, octopuses, and cuttlefish. The name "Decapodiformes" comes from the Greek words "deca," meaning ten, and "podos," meaning foot, referring to the fact that these animals have ten limbs.

However, it is worth noting that within Decapodiformes, octopuses are an exception as they only have eight arms. The other members of this order, such as squids and cuttlefish, have ten appendages, which are used for locomotion, feeding, and sensory perception.

Decapodiformes species are known for their complex behaviors, sophisticated communication systems, and remarkable adaptations that enable them to thrive in a variety of marine habitats. They play important ecological roles as both predators and prey in the ocean food chain.

ErбB-3, also known as HER3 or EGFR3, is a type of receptor tyrosine kinase (RTK) that belongs to the ErbB family of receptors. It is a single-pass transmembrane protein composed of an extracellular ligand-binding domain, a transmembrane region, and an intracellular tyrosine kinase domain.

ErбB-3 plays a crucial role in regulating various cellular processes such as proliferation, differentiation, survival, and migration. However, unlike other ErbB receptors, ErbB-3 lacks intrinsic tyrosine kinase activity due to the presence of several mutations in its kinase domain. Therefore, it requires heterodimerization with other ErbB family members, such as ErbB2 or ErbB4, to become activated and initiate downstream signaling pathways.

The primary ligand for ErbB-3 is neuregulin 1 (NRG1), which binds to the extracellular domain of ErbB-3 and induces its dimerization with other ErbB receptors. This leads to the activation of several downstream signaling pathways, including the PI3K/Akt and MAPK pathways, which promote cell survival, proliferation, and migration.

Abnormal regulation of ErbB-3 has been implicated in various human cancers, such as breast, ovarian, lung, and colon cancer. Overexpression or mutations in ErbB-3 have been shown to contribute to tumor growth, progression, and resistance to therapy. Therefore, targeting ErbB-3 is an active area of research for the development of novel cancer therapies.

The neuromuscular junction (NMJ) is the specialized synapse or chemical communication point, where the motor neuron's nerve terminal (presynaptic element) meets the muscle fiber's motor end plate (postsynaptic element). This junction plays a crucial role in controlling muscle contraction and relaxation.

At the NMJ, the neurotransmitter acetylcholine is released from the presynaptic nerve terminal into the synaptic cleft, following an action potential. Acetylcholine then binds to nicotinic acetylcholine receptors on the postsynaptic membrane of the muscle fiber, leading to the generation of an end-plate potential. If sufficient end-plate potentials are generated and summate, they will trigger an action potential in the muscle fiber, ultimately causing muscle contraction.

Dysfunction at the neuromuscular junction can result in various neuromuscular disorders, such as myasthenia gravis, where autoantibodies attack acetylcholine receptors, leading to muscle weakness and fatigue.

Cell adhesion molecules (CAMs) are a type of protein that mediates the attachment or binding of cells to their surrounding extracellular matrix or to other cells. Neuronal cell adhesion molecules (NCAMs) are a specific subtype of CAMs that are primarily expressed on neurons and play crucial roles in the development, maintenance, and function of the nervous system.

NCAMs are involved in various processes such as cell recognition, migration, differentiation, synaptic plasticity, and neural circuit formation. They can interact with other NCAMs or other types of CAMs to form homophilic or heterophilic bonds, respectively. The binding of NCAMs can activate intracellular signaling pathways that regulate various cellular responses.

NCAMs are classified into three major families based on their molecular structure: the immunoglobulin superfamily (Ig-CAMs), the cadherin family, and the integrin family. The Ig-CAMs include NCAM1 (also known as CD56), which is a glycoprotein with multiple extracellular Ig-like domains and intracellular signaling motifs. The cadherin family includes N-cadherin, which mediates calcium-dependent cell-cell adhesion. The integrin family includes integrins such as α5β1 and αVβ3, which mediate cell-matrix adhesion.

Abnormalities in NCAMs have been implicated in various neurological disorders, including schizophrenia, Alzheimer's disease, and autism spectrum disorder. Therefore, understanding the structure and function of NCAMs is essential for developing therapeutic strategies to treat these conditions.

Denervation is a medical term that refers to the loss or removal of nerve supply to an organ or body part. This can occur as a result of surgical intervention, injury, or disease processes that damage the nerves leading to the affected area. The consequences of denervation depend on the specific organ or tissue involved, but generally, it can lead to changes in function, sensation, and muscle tone. For example, denervation of a skeletal muscle can cause weakness, atrophy, and altered reflexes. Similarly, denervation of an organ such as the heart can lead to abnormalities in heart rate and rhythm. In some cases, denervation may be intentional, such as during surgical procedures aimed at treating chronic pain or spasticity.

Cell movement, also known as cell motility, refers to the ability of cells to move independently and change their location within tissue or inside the body. This process is essential for various biological functions, including embryonic development, wound healing, immune responses, and cancer metastasis.

There are several types of cell movement, including:

1. **Crawling or mesenchymal migration:** Cells move by extending and retracting protrusions called pseudopodia or filopodia, which contain actin filaments. This type of movement is common in fibroblasts, immune cells, and cancer cells during tissue invasion and metastasis.
2. **Amoeboid migration:** Cells move by changing their shape and squeezing through tight spaces without forming protrusions. This type of movement is often observed in white blood cells (leukocytes) as they migrate through the body to fight infections.
3. **Pseudopodial extension:** Cells extend pseudopodia, which are temporary cytoplasmic projections containing actin filaments. These protrusions help the cell explore its environment and move forward.
4. **Bacterial flagellar motion:** Bacteria use a whip-like structure called a flagellum to propel themselves through their environment. The rotation of the flagellum is driven by a molecular motor in the bacterial cell membrane.
5. **Ciliary and ependymal movement:** Ciliated cells, such as those lining the respiratory tract and fallopian tubes, have hair-like structures called cilia that beat in coordinated waves to move fluids or mucus across the cell surface.

Cell movement is regulated by a complex interplay of signaling pathways, cytoskeletal rearrangements, and adhesion molecules, which enable cells to respond to environmental cues and navigate through tissues.

No FAQ available that match "schwann cells"

No images available that match "schwann cells"