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)
Filaments 7-11 nm in diameter found in the cytoplasm of all cells. Many specific proteins belong to this group, e.g., desmin, vimentin, prekeratin, decamin, skeletin, neurofilin, neurofilament protein, and glial fibrillary acid protein.
Cytoplasmic filaments intermediate in diameter (about 10 nanometers) between the microfilaments and the microtubules. They may be composed of any of a number of different proteins and form a ring around the cell nucleus.
The directed transport of ORGANELLES and molecules along nerve cell AXONS. Transport can be anterograde (from the cell body) or retrograde (toward the cell body). (Alberts et al., Molecular Biology of the Cell, 3d ed, pG3)
Nerve fibers that are capable of rapidly conducting impulses away from the neuron cell body.
A prion disease found exclusively among the Fore linguistic group natives of the highlands of NEW GUINEA. The illness is primarily restricted to adult females and children of both sexes. It is marked by the subacute onset of tremor and ataxia followed by motor weakness and incontinence. Death occurs within 3-6 months of disease onset. The condition is associated with ritual cannibalism, and has become rare since this practice has been discontinued. Pathologic features include a noninflammatory loss of neurons that is most prominent in the cerebellum, glial proliferation, and amyloid plaques. (From Adams et al., Principles of Neurology, 6th ed, p773)
Subcellular structures found in nerve cell bodies and DENDRITES. They consist of granular endoplasmic reticulum (ENDOPLASMIC RETICULUM, ROUGH) and RIBOSOMES.
The network of filaments, tubules, and interconnecting filamentous bridges which give shape, structure, and organization to the cytoplasm.
The delicate interlacing threads, formed by aggregations of neurofilaments and neurotubules, coursing through the CYTOPLASM of the body of a NEURON and extending from one DENDRITE into another or into the AXON.
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 cylindrical column of tissue that lies within the vertebral canal. It is composed of WHITE MATTER and GRAY MATTER.
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.
An intermediate filament protein found in most differentiating cells, in cells grown in tissue culture, and in certain fully differentiated cells. Its insolubility suggests that it serves a structural function in the cytoplasm. MW 52,000.
The sum of the weight of all the atoms in a molecule.
An intermediate filament protein found only in glial cells or cells of glial origin. MW 51,000.
Type III intermediate filament proteins expressed mainly in neurons of the peripheral and CENTRAL NERVOUS SYSTEMS. Peripherins are implicated in neurite elongation during development and axonal regeneration after injury.
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.
A serine-threonine kinase that plays important roles in CELL DIFFERENTIATION; CELL MIGRATION; and CELL DEATH of NERVE CELLS. It is closely related to other CYCLIN-DEPENDENT KINASES but does not seem to participate in CELL CYCLE regulation.
The 2nd cranial nerve which conveys visual information from the RETINA to the brain. The nerve carries the axons of the RETINAL GANGLION CELLS which sort at the OPTIC CHIASM and continue via the OPTIC TRACTS to the brain. The largest projection is to the lateral geniculate nuclei; other targets include the SUPERIOR COLLICULI and the SUPRACHIASMATIC NUCLEI. Though known as the second cranial nerve, it is considered part of the CENTRAL NERVOUS SYSTEM.
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.
The introduction of a phosphoryl group into a compound through the formation of an ester bond between the compound and a phosphorus moiety.
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.
Test for tissue antigen using either a direct method, by conjugation of antibody with fluorescent dye (FLUORESCENT ANTIBODY TECHNIQUE, DIRECT) or an indirect method, by formation of antigen-antibody complex which is then labeled with fluorescein-conjugated anti-immunoglobulin antibody (FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT). The tissue is then examined by fluorescence microscopy.
A microtubule subunit protein found in large quantities in mammalian brain. It has also been isolated from SPERM FLAGELLUM; CILIA; and other sources. Structurally, the protein is a dimer with a molecular weight of approximately 120,000 and a sedimentation coefficient of 5.8S. It binds to COLCHICINE; VINCRISTINE; and VINBLASTINE.
Histochemical localization of immunoreactive substances using labeled antibodies as reagents.
Cysteine proteinase found in many tissues. Hydrolyzes a variety of endogenous proteins including NEUROPEPTIDES; CYTOSKELETAL PROTEINS; proteins from SMOOTH MUSCLE; CARDIAC MUSCLE; liver; platelets; and erythrocytes. Two subclasses having high and low calcium sensitivity are known. Removes Z-discs and M-lines from myofibrils. Activates phosphorylase kinase and cyclic nucleotide-independent protein kinase. This enzyme was formerly listed as EC 3.4.22.4.
Changes in the amounts of various chemicals (neurotransmitters, receptors, enzymes, and other metabolites) specific to the area of the central nervous system contained within the head. These are monitored over time, during sensory stimulation, or under different disease states.
The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.
Electrophoresis in which a polyacrylamide gel is used as the diffusion medium.
Major constituent of the cytoskeleton found in the cytoplasm of eukaryotic cells. They form a flexible framework for the cell, provide attachment points for organelles and formed bodies, and make communication between parts of the cell possible.
Techniques for removal by adsorption and subsequent elution of a specific antibody or antigen using an immunosorbent containing the homologous antigen or antibody.
Study of intracellular distribution of chemicals, reaction sites, enzymes, etc., by means of staining reactions, radioactive isotope uptake, selective metal distribution in electron microscopy, or other methods.
A common neoplasm of early childhood arising from neural crest cells in the sympathetic nervous system, and characterized by diverse clinical behavior, ranging from spontaneous remission to rapid metastatic progression and death. This tumor is the most common intraabdominal malignancy of childhood, but it may also arise from thorax, neck, or rarely occur in the central nervous system. Histologic features include uniform round cells with hyperchromatic nuclei arranged in nests and separated by fibrovascular septa. Neuroblastomas may be associated with the opsoclonus-myoclonus syndrome. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2099-2101; Curr Opin Oncol 1998 Jan;10(1):43-51)
The resection or removal of the nerve to an organ or part. (Dorland, 28th ed)
Loss of functional activity and trophic degeneration of nerve axons and their terminal arborizations following the destruction of their cells of origin or interruption of their continuity with these cells. The pathology is characteristic of neurodegenerative diseases. Often the process of nerve degeneration is studied in research on neuroanatomical localization and correlation of the neurophysiology of neural pathways.
Neurons of the innermost layer of the retina, the internal plexiform layer. They are of variable sizes and shapes, and their axons project via the OPTIC NERVE to the brain. A small subset of these cells act as photoreceptors with projections to the SUPRACHIASMATIC NUCLEUS, the center for regulating CIRCADIAN RHYTHM.
Neurons which activate MUSCLE CELLS.
Domesticated bovine animals of the genus Bos, usually kept on a farm or ranch and used for the production of meat or dairy products or for heavy labor.
Sites on an antigen that interact with specific antibodies.
Factors which enhance the growth potentialities of sensory and sympathetic nerve cells.
Antibodies produced by a single clone of cells.
The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.
A degenerative disease of the BRAIN characterized by the insidious onset of DEMENTIA. Impairment of MEMORY, judgment, attention span, and problem solving skills are followed by severe APRAXIAS and a global loss of cognitive abilities. The condition primarily occurs after age 60, and is marked pathologically by severe cortical atrophy and the triad of SENILE PLAQUES; NEUROFIBRILLARY TANGLES; and NEUROPIL THREADS. (From Adams et al., Principles of Neurology, 6th ed, pp1049-57)
The property of antibodies which enables them to react with some ANTIGENIC DETERMINANTS and not with others. Specificity is dependent on chemical composition, physical forces, and molecular structure at the binding site.
Slender, cylindrical filaments found in the cytoskeleton of plant and animal cells. They are composed of the protein TUBULIN and are influenced by TUBULIN MODULATORS.
A class of fibrous proteins or scleroproteins that represents the principal constituent of EPIDERMIS; HAIR; NAILS; horny tissues, and the organic matrix of tooth ENAMEL. Two major conformational groups have been characterized, alpha-keratin, whose peptide backbone forms a coiled-coil alpha helical structure consisting of TYPE I KERATIN and a TYPE II KERATIN, and beta-keratin, whose backbone forms a zigzag or pleated sheet structure. alpha-Keratins have been classified into at least 20 subtypes. In addition multiple isoforms of subtypes have been found which may be due to GENE DUPLICATION.
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
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.
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.
Elements of limited time intervals, contributing to particular results or situations.
Immunologic techniques based on the use of: (1) enzyme-antibody conjugates; (2) enzyme-antigen conjugates; (3) antienzyme antibody followed by its homologous enzyme; or (4) enzyme-antienzyme complexes. These are used histologically for visualizing or labeling tissue specimens.

Nerve terminal damage by beta-bungarotoxin: its clinical significance. (1/1047)

We report here original data on the biological basis of prolonged neuromuscular paralysis caused by the toxic phospholipase A2 beta-bungarotoxin. Electron microscopy and immunocytochemical labeling with anti-synaptophysin and anti-neurofilament have been used to show that the early onset of paralysis is associated with the depletion of synaptic vesicles from the motor nerve terminals of skeletal muscle and that this is followed by the destruction of the motor nerve terminal and the degeneration of the cytoskeleton of the intramuscular axons. The postjunctional architecture of the junctions were unaffected and the binding of fluorescein-isothiocyanate-conjugated alpha-bungarotoxin to acetylcholine receptor was not apparently affected by exposure to beta-bungarotoxin. The re-innervation of the muscle fiber was associated by extensive pre- and post-terminal sprouting at 3 to 5 days but was stable by 7 days. Extensive collateral innervation of adjacent muscle fibers was a significant feature of the re-innervated neuromuscular junctions. These findings suggest that the prolonged and severe paralysis seen in victims of envenoming bites by kraits (elapid snakes of the genus Bungarus) and other related snakes of the family Elapidae is caused by the depletion of synaptic vesicles from motor nerve terminals and the degeneration of the motor nerve terminal and intramuscular axons.  (+info)

ELAV tumor antigen, Hel-N1, increases translation of neurofilament M mRNA and induces formation of neurites in human teratocarcinoma cells. (2/1047)

Human ELAV proteins are implicated in cell growth and differentiation via regulation of mRNA expression in the cytoplasm. In human embryonic teratocarcinoma (hNT2) cells transfected with the human neuronal ELAV-like protein, Hel-N1, neurites formed, yet cells were not terminally differentiated. Cells in which neurite formation was associated with Hel-N1 overexpression, also expressed increased levels of endogenous neurofilament M (NF-M) protein, which distributed along the neurites. However, steady-state levels of NF-M mRNA remained similar whether or not hNT2 cells were transfected with Hel-N1. These findings suggest that turnover of NF-M mRNA was not affected by Hel-N1 expression, despite the fact that Hel-N1 can bind to the 3' UTR of NF-M mRNA and was found directly associated with NF-M mRNA in transfected cells. Analysis of the association of NF-M mRNA with the translational apparatus in Hel-N1 transfectants showed nearly complete recruitment to heavy polysomes, indicating that Hel-N1 caused an increase in translational initiation. Our results suggest that the stability and/or translation of ARE-containing mRNAs can be regulated independently by the ELAV protein, Hel-N1, depending upon sequence elements in the 3' UTRs and upon the inherent turnover rates of the mRNAs that are bound to Hel-N1 in vivo.  (+info)

Deamidation and isoaspartate formation in smeared tau in paired helical filaments. Unusual properties of the microtubule-binding domain of tau. (3/1047)

An extensive loss of a selected population of neurons in Alzheimer's disease is closely related to the formation of paired helical filaments (PHFs). The most striking characteristic of PHFs upon Western blotting is their smearing. According to a previously described protocol (Morishima-Kawashima, M., Hasegawa, M., Takio, K., Suzuki, M., Titani, K., and Ihara, Y. (1993) Neuron 10, 1151-1160), smeared tau was purified, and its peptide map was compared with that of soluble (normal) tau. A CNBr fragment from soluble tau (CN5; residues 251-419 according to the 441-residue isoform) containing the microtubule-binding domain migrated at 15 and 18 kDa on SDS-polyacrylamide gel electrophoresis, whereas that from smeared tau exhibited two larger, unusually broad bands at approximately 30 and approximately 45 kDa, presumably representing dimers and trimers of CN5. In the peptide map of smeared tau-derived CN5, distinct peaks eluting at unusual locations were noted. Amino acid sequence and mass spectrometric analyses revealed that these distinct peptides bear isoaspartate at Asn-381 and Asp-387. Because no unusual peptides other than aspartyl or isoaspartyl peptide were found in the digests of smeared tau-derived CN5, it is likely that site-specific deamidation and isoaspartate formation are involved in its dimerization and trimerization and thus in PHF formation in vivo.  (+info)

Aberrant neurofilament phosphorylation in sensory neurons of rats with diabetic neuropathy. (4/1047)

Aberrant neurofilament phosphorylation occurs in many neurodegenerative diseases, and in this study, two animal models of type 1 diabetes--the spontaneously diabetic BB rat and the streptozocin-induced diabetic rat--have been used to determine whether such a phenomenon is involved in the etiology of the symmetrical sensory polyneuropathy commonly associated with diabetes. There was a two- to threefold (P < 0.05) elevation of neurofilament phosphorylation in lumbar dorsal root ganglia (DRG) of diabetic rats that was localized to perikarya of medium to large neurons using immunocytochemistry. Additionally, diabetes enhanced neurofilament M phosphorylation by 2.5-fold (P < 0.001) in sural nerve of BB rats. Neurofilaments are substrates of the mitogen-activated protein kinase (MAPK) family, which includes c-jun NH2-terminal kinase (JNK) or stress-activated protein kinase (SAPK1) and extracellular signal-regulated kinases (ERKs) 1 and 2. Diabetes induced a significant three- to fourfold (P < 0.05) increase in phosphorylation of a 54-kDa isoform of JNK in DRG and sural nerve, and this correlated with elevated c-Jun and neurofilament phosphorylation. In diabetes, ERK phosphorylation was also increased in the DRG, but not in sural nerve. Immunocytochemistry showed that JNK was present in sensory neuron perikarya and axons. Motoneuron perikarya and peroneal nerve of diabetic rats showed no evidence of increased neurofilament phosphorylation and failed to exhibit phosphorylation of JNK. It is hypothesized that in sensory neurons of diabetic rats, aberrant phosphorylation of neurofilament may contribute to the distal sensory axonopathy observed in diabetes.  (+info)

Overexpression of alpha-internexin causes abnormal neurofilamentous accumulations and motor coordination deficits in transgenic mice. (5/1047)

alpha-Internexin is the first neuronal intermediate filament (IF) protein expressed in postmitotic neurons of the developing nervous system. In the adult, its expression is restricted to mature neurons in the CNS. To study the potential role of alpha-internexin in neurodegeneration, we have generated transgenic mice that overexpress rat alpha-internexin. The total levels of alpha-internexin expressed in the hemizygous and homozygous transgenic mice were approximately 2 and approximately 3 times the normal level, respectively. Overexpression of alpha-internexin resulted in the formation of cerebellar torpedoes as early as 1 month of age. These torpedoes are abnormal swellings of Purkinje cell axons that are usually seen in neurodegenerative diseases involving the cerebellum. EM studies showed accumulations of high levels of IFs and abnormal organelles in the torpedoes and soma of Purkinje cells, as well as in the large pyramidal neurons of the neocortex and in the ventral anterior and posteromedial nuclei of the thalamus. Behavioral tests demonstrate that these mice have a deficit in motor coordination as early as 3 months of age, consistent with the morphological neuronal changes. Our data further demonstrate that the neurofilamentous inclusions also lead to progressive loss of neurons in the aged transgenic mice. The motor coordination deficit and the loss of neurons are transgene dosage-dependent. These data yield direct evidence that high levels of misaccumulated neuronal IFs lead to neuronal dysfunction, progressive neurodegeneration, and ultimate loss of neurons. Moreover, the degrees of neuronal dysfunction and degeneration are proportional to the levels of misaccumulated neuronal IFs.  (+info)

Development of the chick olfactory nerve. (6/1047)

Gonadotropin releasing hormone (GnRH) is produced and secreted by neurons dispersed throughout the septal-preoptic and anterior hypothalamic areas in adult birds and mammals. These neurons, essential for a functional brain-pituitary-gonadal axis, differentiate in the olfactory placode, the superior aspect of which forms the olfactory epithelium. To reach their final placement within the brain, GnRH neurons migrate out of the epithelium and along the olfactory nerve to the CNS. This nerve is essential for the entrance of GnRH neurons into the CNS. Due to the importance of the nerve for the proper migration of these neurons, we have used immunocytochemistry, DiI labeling and 1 microm serial plastic-embedded sections to characterize the nerve's earliest development in the embryonic chick (stages 17-21). Initially (stage 17) the zone between the placode and prosencephalon is a cellular mass contiguous with the placode. This cluster, known as epithelioid cells, is positive for some but not all neuronal markers studied. The epithelium itself is negative for all neuronal and glial markers at this early stage. By stage 18, the first neurites emerge from the epithelium; this was confirmed at stage 19 by examination of serial 1 microm plastic sections. There is sequential acquisition of immunoreactivity to neuronal markers from stage 18 to 21. The glial component of the nerve appears at stage 21. Axons originating from epithelium, extend to the border of the CNS as confirmed by DiI labeling at stage 21. Small fascicles have entered the CNS at this stage. As previously reported, GnRH neurons begin their migration between stages 20-21 and have also arrived at the border of the brain at stage 21. Despite the penetration of neurites from the olfactory nerve into the CNS, GnRH neurons pause at the nerve-brain junction until stage 29 (2 1/2 days later) before entering the brain. Subsequent studies will examine the nature of the impediment to continued GnRH neuronal migration.  (+info)

Claudin-11/OSP-based tight junctions of myelin sheaths in brain and Sertoli cells in testis. (7/1047)

Members of the newly identified claudin gene family constitute tight junction (TJ) strands, which play a pivotal role in compartmentalization in multicellular organisms. We identified oligodendrocyte-specific protein (OSP) as claudin-11, a new claudin family member, due to its sequence similarity to claudins as well as its ability to form TJ strands in transfected fibroblasts. Claudin-11/OSP mRNA was expressed in the brain and testis. Immunofluorescence microscopy with anti-claudin-11/OSP polyclonal antibody (pAb) and anti-neurofilament mAb revealed that in the brain claudin-11/OSP-positive linear structures run in a gentle spiral around neurofilament-positive axons. At the electron microscopic level, these linear structures were identified as the so-called interlamellar strands in myelin sheaths of oligodendrocytes. In testis, well-developed TJ strands of Sertoli cells were specifically labeled with anti-claudin-11/OSP pAb both at immunofluorescence and electron microscopic levels. These findings indicated that the interlamellar strands of oligodendrocyte myelin sheaths can be regarded as a variant of TJ strands found in many other epithelial cells, and that these strands share a specific claudin species, claudin-11/OSP, with those in Sertoli cells to create and maintain the repeated compartments around axons by oligodendrocytes.  (+info)

Activation of mitogen-activated protein kinases (Erk1 and Erk2) cascade results in phosphorylation of NF-M tail domains in transfected NIH 3T3 cells. (8/1047)

Neurofilaments (NFs) are neuron-specific intermediate filaments, and are the major cytoskeletal component in large myelinated axons. Lysine-serine-proline (KSP) repeats in the tail domains of high molecular weight NF proteins (NF-M and NF-H) are extensively phosphorylated in vivo in the axon. This phosphorylation in the tail domain has been postulated to play an important role in mediating neuron-specific properties, including axonal caliber and conduction velocity. Recent studies have shown that the mitogen-activated protein kinases (extracellular signal-regulated kinases, Erk1 and Erk2) phosphorylate KSP motifs in peptide substrates derived from the NF-M and NF-H tail domains in vitro. However, it is not clear whether activation of the mitogen activated protein (MAP) kinase pathway is able to phosphorylate these domains in vivo. To answer this question, a constitutively active form of mitogen-activated Erk activating kinase (MEK1) was cotransfected with an NF-M expression construct into NIH 3T3 cells. The activated mutant, but not the dominant negative mutant, induced phosphorylation of NF-M. In addition, it was shown that epidermal growth factor, which induces the MAP kinase cascade in NIH 3T3 cells, also activated endogenous Erk1 and Erk2 and NF-M tail domain phosphorylation in the transfected cells. These results present direct evidence that in-vivo activation of Erk1 and Erk 2 is sufficient for NF-M tail domain phosphorylation in transfected cells.  (+info)

Human immunodeficiency virus (HIV) is able to enter from the periphery to the central nervous system, triggering neurocognitive impairment which can be asymptomatic, mild cognitive and motor impairment or in a few cases severe dementia. With the availability and efficacy of antiretroviral therapy, HIV patients have a better quality of life. However, HIV-associated neurocognitive disorders (HAND) still prevail in near 50% of the treated patients. In this study, we used ELISA to measure neurofilament light chain (NF-L) and neopterin, as markers of neuronal injury and immune activation, respectively. We used plasma and cerebrospinal (CSF) samples obtained from HIV-seropositive (HIV+) patients (n=35) at different stages of HAND, compared to HIV-seronegative controls (n=10). HIV+ patients were subjected to standard laboratory tests (including viral load and CD4+ cell count) and neuropsychological tests (eight domains). Statistical tests included Mann-Whitney, Kruskal-Wallis and Spearmans ...
1. Recent examination of the hypothesis that distinctly phosphorylated NF-H isoforms exist in different types of neurons revealed that the extent of phosphorylation of the heavy neurofilament...
Neurofilaments (NF) are a key component of the neuronal cytoskeleton, and are responsible for providing structural support to the axon and regulating axon diameter. Mammalian neurofilaments are composed of three major subunits, classified based on molecular weight in SDS-PAGE: light (NF-L), 68-70 kDa; medium (NF-M), 145-160 kDa; and heavy (NF-H), 200-220 kDa. In humans, these proteins are encoded by the NEFL, NEFM, and NEFH genes, respectively. NF-M is also known as neurofilament, medium polypeptide; neurofilament, medium polypeptide 160kDa; NEF3, neurofilament 3, neurofilament triplet M protein, and 160 kDa neurofilament protein. NF-H is also known as neurofilament, heavy polypeptide; neurofilament, heavy polypeptide 200kDa; KIAA0845, neurofilament triplet H protein, and 200 kDa neurofilament protein.. ...
neuroaxonal damage is the pathological substrate of permanent disability in various neurological disorders. ... Here, we review what is known about the structure and function of neurofilaments, discuss analytical aspects and knowledge of age-dependent normal ranges of neurofilaments and provide a comprehensive overview of studies on neurofilament light chain as a marker of axonal injury in different neurological disorders, including multiple sclerosis, neurodegenerative dementia, stroke, traumatic brain injury, amyotrophic lateral sclerosis and Parkinson disease ...
Uphaus T, Bittner S, Groschel S, Steffen F, Muthuraman M, Wasser K, Weber-Kruger M, Zipp F, Wachter R and Groschel K. Stroke. 2019 Sep 20:STROKEAHA119026410. doi: 10.1161/STROKEAHA.119.026410
Our data support that DRE is associated with an expansion of the CD4 Tcell subset in the peripheral blood and with a shift toward a proinflammatory Th17/Th1 CD4 Tcell immune profile. Our results further show that pathological levels of sNfL are more frequent in DRE, supporting a potential neurodegen …
Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/. ...
GENETICS IN MEDICINE | NOVEMBER 26, 2020 Dang Do AN, Sinaii N, Masvekar RR, Baker EH, Thurm AE, Soldatos AG, Bianconi SE, Bielekova B and Porter FD. Genet
The transactive response DNA-binding protein 43 (TDP-43) has been identified as a neurofilament light (NF-L) messenger RNA (mRNA)-binding protein. Abnormally increased levels of TDP-43 are detected in patients with amyotrophic lateral sclerosis and a downregulation of NF-L mRNA. However, links between NF-L and TDP-43 expressions are unclear. In this study, we investigated whether the deficiency of NF-L protein can result in alterations in TDP-43 localization or protein expression and whether this is altered with aging. There was a significant increase in TDP-43 protein levels in the cortex and lumbar spinal cord in 12-month-old NF-L knockout (NF-L KO) mice, compared with wild-type (WT) C57BL/6 mice. However, there was no difference in either the phosphorylation of TDP-43 between WT and NF-L KO mice or the abnormal mislocalization of TDP-43 to the cytoplasm in NF-L KO animals. Our findings suggest that NF-L protein or mRNA may negatively affect the expression of TDP-43 in the central nervous ...
Several recent animal models have clearly demonstrated that axonal NFs are not essential for the survival of small animals (Eyer and Peterson, 1994; Ohara et al., 1993; Zhu et al., 1997). Yet the highly conserved nature of NF proteins suggests that they serve some important function. NFs have long been suspected to help specify the diameter of axons and to support their integrity (Hoffman et al., 1988) and these same animal models (Eyer and Peterson, 1994; Ohara et al., 1993; Zhu et al., 1997) have clearly shown that radial growth is impaired in axons lacking NFs. However, how NFs increase axon caliber and what role individual subunits play in this process remains to be clarified. Myelin-forming cells may also influence axonal diameter through affects on the amount and phosphorylation state of NF subunits (Cole et al., 1994; de Waegh et al., 1992; Hsieh et al., 1994; Nixon et al., 1994; Yin et al., 1998). Thus, NFs may function as effector proteins subject to a complex regulatory cross-talk ...
Research proven, mouse monoclonal NeurofilamentL antibody. Useful as a marker for neurons and neurogenesis. NF-L antibody can also be useful in the diagnostics of neurofilament accumulations seen in many neurological diseases, such as Lou Gehrigs disease or Alzheimers disease. Excellent for immunohistochemistry, immunofluorecence, western blotting and related applications. IF and WB images available.
Conclusions In ALS, increased pNF-H concentration in plasma, serum and CSF appears to be associated with faster disease progression. Factors affecting pNF-H levels or their detection in serum and plasma in relation to disease course may differ from those in CSF. Data raising the possibility that site of ALS onset (bulbar vs spinal) may influence pNF-H levels in peripheral blood seems noteworthy but requires confirmation. These data support further study of pNF-H in CSF, serum and plasma as a potential ALS biomarker.. ...
TY - JOUR. T1 - Cationic lipid-mediated NGF gene transfection increases neurofilament phosphorylation. AU - Yang, K.. AU - Xue, J. J.. AU - Qiu, Y. H.. AU - Perez-Polo, J. R.. AU - Whitson, J.. AU - Faustinella, F.. AU - Kampfl, A.. AU - Zhao, X.. AU - Iwamoto, Y.. AU - Mu, X. S.. AU - Clifton, G.. AU - Hayes, R. L.. PY - 1996/12/31. Y1 - 1996/12/31. N2 - We examined the effect of cationic lipid-mediated gene transfection of nerve growth factor (NGF) in primary septo-hippocampal cell cultures. Rat NGF cDNA was subcloned into a pUC19-based plasmid containing a CMV promoter. Two days after NGF gene transfection in primary cell cultures, ELISA confirmed increases in NGF protein secretion from transfected cells. To study the biological effect of cationic lipid-mediated NGF gene transfection, we analyzed the amount of neurofilament protein from NGF-transfected cell cultures. Western blot and immunohistochemical analyses detected significant increases in the phosphorylated form of neurofilament ...
Biomarkers that can track disease onset and progression in autosomal dominant Alzheimers disease (ADAD) are needed. We investigate whether serum neurofilament light (NfL) concentration is associated with clinical and cerebrospinal fluid (CSF) markers in ADAD. We also evaluate serum NfL differences between clinical groups. Serum NfL was measured cross-sectionally in 60 individuals from ADAD families using an ultrasensitive immunoassay on the Single molecule array (Simoa) platform and longitudinally in an exploratory study in a subset of six mutation carriers. Spearman coefficients assessed associations between serum NfL and relevant measures. Differences between groups were evaluated by Kruskal-Wallis and Mann-Whitney U tests. Forty-two participants were mutation carriers: 22 symptomatic (SMC) and 20 asymptomatic (AMC). Eighteen subjects were non-carriers and cognitively normal (controls (CTR)). Serum NfL correlated with the estimated years from symptoms onset across mutation carriers (rho = 0.75, p | 0
The neurofilaments (NFs), the intermediate filaments of the neuronal cytoskeleton, provide mechanical stability to the cell. NFs are relatively scarce in the neuronal cell body and dendrites and are mainly distributed in the nerve axon. NFs have three types of subunits, NF-L (low), NF-M (medium), and NF-H (heavy). pNF-H contains a heavily phosphorylated carboxyl terminal sidearm domain which helps determine the interfilament spacing distances. pNF-H has high resistance to protease, so pNF-H released from damaged axons does not decompose but remains unchanged in the body.[7] pNF-H is specifically expressed in the neuronal body. Therefore, detection of pNF-H in the blood or cerebrospinal fluid could be a useful biomarker to evaluate neuronal damage.[5] The serum pNF-H level increases after injury and remains high for several days after injury in the spinal cord injury model.[8] In addition, pNF-H has been reported as a useful predictor after spinal cord injury.[3,4,10]. However, the value of ...
purpose. To study the time-dependent effects of elevated intraocular pressure (IOP) on axonal transport and cytoskeleton proteins in the porcine optic nerve head.. methods. Fifteen pigs were used for this study. Rhodamine-β-isothiocyanate was injected into the vitreous of each eye to study axonal transport. IOP in the left eye was elevated to 40 to 45 mm Hg, and IOP in the right eye was maintained between 10 and 15 mm Hg. Cerebrospinal fluid pressure was also continually monitored. IOP was elevated for 3 hours (n = 7) or 12 hours (n = 8) before animal euthanatization. Antibodies to phosphorylated neurofilament heavy (NFHp), phosphorylation-independent neurofilament heavy (NFH), neurofilament light, neurofilament medium (NFM), microtubule, and microtubule-associated protein (MAP) were used to study the axonal cytoskeleton. Confocal microscopy was used to compare axonal transport and cytoskeleton change between control and high IOP eyes in different laminar regions and quadrants of the optic ...
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Description: Neurofilaments are a type of intermediate filament that serve as major elements of the cytoskeleton supporting the axon cytoplasm. They are the most abundant fibrillar components of the axon, being on average 3-10 times more frequent than axonal microtubules. Neurofilaments (10nm in dia.) are built from three intertwined protofibrils which are themselves composed of two tetrameric protofilament complexs of monomeric proteins. The neurofilament triplet proteins (68/70, 160, and 200 kDa) occur in both the central and peripheral nervous system and are usually neuron specific. The 68/70 kDa NF-L protein can self-assemble into a filamentous structure, however the 160 kDa NF-M and 200 kDa NF-H proteins require the presence of the 68/70 kDa NF-L protein to co-assemble. Neuromas, ganglioneuromas, gangliogliomas, ganglioneuroblastomas and neuroblastomas stain positively for neurofilaments. Although typically restricted to neurons, neurofilaments have been detected in paragangliomas and ...
Blood markers indicative of neurodegeneration (neurofilament light chain; NFL), Alzheimers disease amyloid pathology (amyloid-β; Aβ), and neuroinflammation (kynurenine pathway; KP metabolites) have been investigated independently in neurodegenerative diseases. However, the association of these markers of neurodegeneration and AD pathology with neuroinflammation has not been investigated previously. Therefore, the current study examined whether NFL and Aβ correlate with KP metabolites in elderly individuals to provide insight on the association between blood indicators of neurodegeneration and neuroinflammation.Correlations between KP metabolites, measured using liquid chromatography and gas chromatography coupled with mass spectrometry, and plasma NFL and Aβ concentrations, measured using single molecule array (Simoa) assays, were investigated in elderly individuals aged 65-90 years, with normal global cognition (Mini-Mental State Examination Score ≥ 26) from the Kerr Anglican Retirement ...
The test measures the neurofilament light chain (neurofilament), a protein released from damaged brain cells, which has been linked to other neurodegenerative diseases but hasnt been studied in the blood of Huntingtons disease (HD) patients before.. The team, led by scientists at the UCL Huntingtons Disease Centre working with colleagues in Sweden, the USA, Canada, France and the Netherlands, measured neurofilament levels in blood samples from the TRACK-HD study, an international project that followed 366 volunteers for three years. They found that levels of the brain protein were increased throughout the course of HD - even in carriers of the HD genetic mutation who were many years from showing symptoms of the disease. HD mutation carriers had neurofilament concentrations that were 2.6 times that of the control participants, and the level rose throughout the disease course from premanifest to stage 2 disease.. In the group who had no symptoms at the start of the study, the level of ...
Anti-160 kD Neurofilament Medium antibody (ab39371) has been cited in 2 publications. References for Human, Mouse in IHC-Fr, IHC-P
Essential tremor (ET) is one of the most common neurological diseases. A basic understanding of its neuropathology is now emerging. Aside from Purkinje cell loss, a prominent finding is an abundance of torpedoes (rounded swellings of Purkinje cell axons). Such swellings often result from the mis-accumulation of cell constituents. Identifying the basic nature of these accumulations is an important step in understanding the underlying disease process. Torpedoes, only recently identified in ET, have not yet been characterized ultrastructurally. Light and electron microscopy were used to characterize the structural constituents of torpedoes in ET. Formalin-fixed cerebellar cortical tissue from four prospectively collected ET brains was sectioned and immunostained with a monoclonal phosphorylated neurofilament antibody (SMI-31, Covance, Emeryville, CA). Using additional sections from three ET brains, torpedoes were assessed using electron microscopy. Immunoreactivity for phosphorylated neurofilament protein
Objectives Biomarkers for the diagnosis of motoneuron diseases (MND) are urgently needed to improve the diagnostic pathway, patient stratification and monitoring. The aim of this study was to validate candidate markers for MND in cerebrospinal fluid (CSF) and specify cut-offs based on large patient cohorts by especially considering patients who were seen under the initial differential diagnosis (MND mimics). Methods In a prospective study, we investigated CSF of 455 patients for neurofilament light chain (NfL), phosphorylated heavy chain (pNfH), tau protein (Tau) and phospho-tau protein (pTau). Analysed cohorts included patients with apparently sporadic and familial amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS) (MND, n=253), MND mimics (n=85) and neurological control groups. Cut-off values were specified, and diagnostic performance and correlation with progression were analysed. Results Nfs were significantly higher in the MND group compared to the control groups, ...
BACKGROUND: The immune response in Alzheimers disease (AD) involves activation of microglia which may remove amyloid-β (Aβ). However, overproduction of inflammatory compounds may exacerbate neural damage in AD. AD pathology accumulates years before diagnosis, yet the extent to which neuroinflammation is involved in the earliest disease stages is unknown. OBJECTIVE: To determine whether neuroinflammation exacerbates neural damage in preclinical AD. METHODS: We utilized cerebrospinal fluid (CSF) and magnetic resonance imaging collected in 192 asymptomatic late-middle-aged adults (mean age = 60.98 years). Neuroinflammatory markers chitinase-3-like protein 1 (YKL-40) and monocyte chemoattractant protein-1 (MCP-1) in CSF were utilized as markers of neuroinflammation. Neural cell damage was assessed using CSF neurofilament light chain protein (NFL), CSF total tau (T-Tau), and neural microstructure assessed with diffusion tensor imaging (DTI). With regard to AD pathology, CSF Aβ 42 and tau ...
The therapeutic landscape of MS is rapidly evolving due to the development of novel, highly specific immunomodulatory therapies. An optimal stratification of MS patients is still challenging due to heterogenic disease courses and therapy responses. We explore emerging biomarkers indicating disease activitiy and neurodegenerative processes such as neurofilament light chains as a marker of axonal damage in MS patients. Our research group is part of the Focus Program Translational Neuroscience (FTN), the Research Center for Immunotherapy (FZI) and the Rhine-Main Neuroscience Network (rmn2). ...
A study from the National Institutes of Health confirms that neurofilament light chain as a blood biomarker can detect brain injury and predict recovery in multiple groups, including professional hockey players with acute ...
Measuring blood neurofilament light chain (NfL) levels may help distinguish Parkinsons disease (PD) from atypical parkinsonian disorders (APD), according to a study published onlinein Neurology.
The diagnostic and classificatory performances of all combinations of three core (amyloid β peptide [i.e., Aβ1-42], total tau [t-tau], and phosphorylated tau) and three novel (neurofilament light chain protein, neurogranin, and YKL-40) cerebrospinal fluid biomarkers of neurodegeneration were compared among individuals with mild cognitive impairment (n = 41), Alzheimers disease dementia (ADD; Read & Research Alzheimers More. ...
Serum Neurofilament Light Chain Levels in Patients With Presymptomatic Multiple Sclerosis Kjetil Bjornevik, MD, PhD; Kassandra L. Munger, ScD; Marianna Cortese,MD, PhD; Christian Barro, MD; Brian C. Healy; DavidW. Niebuhr, MD; Ann I. Scher, PhD; Jens Kuhle,MD, PhD; Alberto Ascherio,MD, DrPH JAMA Neurol. doi:10.1001/jam
Balastik, M.; Ferraguti, F.; Pires da Silva, A.; Lee, T. H.; Alvarez-Bolado, G.; Lu, K. P.; Gruss, P.: Deficiency in ubiquitin ligase TRIM2 causes accumulation of neurofilament light chain and neurodegeneration. Proceedings of the National Academy of Sciences of the United States of America 105 (33), pp. 12016 - 12021 (2008 ...
Balastik, M.; Ferraguti, F.; Pires da Silva, A.; Lee, T. H.; Alvarez-Bolado, G.; Lu, K. P.; Gruss, P.: Deficiency in ubiquitin ligase TRIM2 causes accumulation of neurofilament light chain and neurodegeneration. Proceedings of the National Academy of Sciences of the United States of America 105 (33), S. 12016 - 12021 (2008 ...
Despite aggressive treatment regimes, glioma remains a largely fatal disease. 146062-49-9 supplier be a proximal objective in the search for more effective cancer treatments. Previously, we demonstrated that intermediate filament proteins contain short motifs that bind unpolymerized tubulin and that 24-amino acid peptides encompassing these tubulin-binding sites (TBS) maintain tubulin-binding capacity. We also demonstrated that a TBS derived from the neurofilament light subunit (neurofilament light (NFL)-TBS.40-63) inhibited tubulin polymerization where it 146062-49-9 supplier led to disruption of their microtubule network and reduced their viability.20 Here, we compared diverse glioma cell lines with various normal cell types for their capacity to internalize NFL-TBS.40-63 peptide exposure. In contrast, a markedly enhanced Rabbit Polyclonal to OR4C6 ability to internalize the peptide was a prominent feature shared among multiple glioma cell lines, and such internalization was accompanied by ...
Neurofilaments form structural networks in neurons and are transported from the neuronal cell body (the site of synthesis) into the axons via a process known as slow axonal transport. Using neurofilament subunits tagged with a fluorophore, Ackerley et al. show that glutamate, a neurotransmitter which at high concentrations leads to excitotoxicity, can alter neurofilament transport. Glutamate slowed neurofilament transport, most probably due to stimulation of mitogen-activated protein kinases, which are capable of phosphorylating neurofilament subunits. This observation provides a mechanistic link between excitotoxicity and neurofilament accumulation associated with neurodegenerative disorders such as Parkinsons disease and amyotrophic lateral sclerosis. - SMH. J. Cell Biol. 150, 165 (2000).. ...
マウス・モノクローナル抗体 ab74592 交差種: Ms,Rat,Rb,Cat,Hu 適用: WB,IHC-P…68kDa Neurofilament抗体一覧…画像、プロトコール、文献などWeb上の情報が満載のアブカムの Antibody…
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The comparison of ZG versus ZF transcriptomes has revealed several unsuspected genes many-fold upregulated in human ZG. NEFM was the fourth most upregulated gene in ZG.21 Moreover, its expression was ≈4-fold higher in ZG-like versus ZF-like APAs.22 Our previous functional studies of 2 of the ZG-specific genes, LGR5 and DACH1, showed that they inhibit aldosterone secretion and proliferation. This surprising finding, together with their absence from upregulated genes in rodent ZG26 and patchiness of aldosterone synthase in human adrenal, was interpreted as an adaptive mechanism to high-salt intake in Western diet.20,21. NEFM encodes one of the subunits forming the neurofilament, the type IV intermediate filament of mature neurons, one of the most abundant proteins in the nervous system. Neurofilament subunits perform extracytoskeletal roles in the neuronal synapses as receptor-interacting proteins. For instance, NEFL interacts directly with one of the subunits of the N-methyl-d-aspartate ...
p,The neurofilament light subunit (NF-L) binds to myosin Va (Myo Va) in neurons but the sites of interaction and functional significance are not clear. We show by deletion analysis that motor domain of Myo Va binds to the NF-L rod domain that forms the NF backbone. Loss of NF-L and Myo Va binding from axons significantly reduces the axonal content of ER, and redistributes ER to the periphery of axon. Our data are consistent with a novel function for NFs as a scaffold in axons for maintaining the content and proper distribution of vesicular organelles, mediated in part by Myo Va. Based on observations that the Myo Va motor domain binds to intermediate filament (IF) proteins of several classes, Myo Va interactions with IFs may serve similar roles in organizing organelle topography in different cell types.,/p,. ...
Finding biomarkers that reflect the amount of peripheral nerve damage (peripheral neuropathies) and that the biomarker will quickly drop in value in response to to effective treatment are desired goals. The tools we need for developing biomarkers for equine neurodegenerative diseases are not available. These tools include a laboratory model for each neurodegenerative disease, putative treatments, and a money bin.. There is an alternate path leading to biomarker development and that is the horizon we are chasing. The biomarker quest project identifies natural cases of disease with neurodegeneration followed by evaluating the data from those cases. Sifting through the data is a process of eliminating the negative, selecting the positive, and interpreting the in-between. I hear a jingle in there somewhere! Generally diseases follow a typical course, or pathogenesis. Interpreting enough cases points toward the direction we should take and where to concentrate our assets. Often clues to a direction ...
Alzheimers disease, Amyloid-beta, Neurofilament light, Neurogranin, YKL-40, Cognition, Cerebrospinal fluid, APOE, MILD COGNITIVE IMPAIRMENT, NEUROFILAMENT LIGHT, NEUROGRANIN, PROTEIN, PATHOLOGY, TAU, DEGENERATION, ASSOCIATION, ...
Key Points: QuestionHow do levels of neurofilament light in cerebrospinal fluid (cNfL) compare between neurological conditions and with healthy controls?. Findings: Among 10 059 individuals in this systematic review and meta-analysis, cNfL was elevated in most neurological conditions compared with healthy controls, and the magnitude of the increase varies extensively. Although cNfL overlaps between most clinically similar conditions, its distribution did not overlap in frontotemporal dementia and other dementias or in Parkinson disease and atypical parkinsonian syndromes.. Meaning: The cNfL is a marker of neuronal damage and may be useful to differentiate some clinically similar conditions, such as frontotemporal dementia from Alzheimer disease and Parkinson disease from atypical parkinsonian syndromes.. This systematic review and meta-analysis assesses the associations of age, sex, and diagnosis with neurofilament light in cerebrospinal fluid and evaluates its potential in discriminating ...
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Alterations occurring in nerve proteins of transected nerves were studied in rat sciatic nerves using polyclonal and monoclonal antibodies to identify and monitor neurofilament (NF) epitopes among nerve proteins following their electrophoresis and transfer to nitrocellulose paper. Immunoblot methods identified NF epitopes in NF triplet proteins (Mr 200,000, 150,000, and 68,000) and in NF nontriplet proteins (all other immunobands below Mr 200,000 and above Mr 40,000). NF triplet and nontriplet proteins were Triton-insoluble in both untransected and transected nerves. Extensive loss of NF triplet and most nontriplet proteins occurred during the 24-48-h period following nerve transection and was attributed to proteolytic degradation. Loss of protease-labile NF proteins led to a markedly reduced level of NF immunoreactivity in 2-day transected nerve. NF proteins which survived the 2-day posttransectional period were considered to represent protease-stable NF fragments. These fragments persisted in
|p|Neurofilaments (NF) are approximately 10 nanometer intermediate filaments found in neurons. They are a major component of the neuronal cytoskeleton, and function primarily to provide structural support for the axon and to regulate the axon diameter. There are three major NF subunits, and the name
The effect of nerve growth factor (NGF) on the expression of neurofilament and Thy-1 genes in rat PC12 pheochromocytoma cells was examined at both the transcriptional and post-transcriptional levels. Addition of NGF to cultured PC12 cells produced increases in mRNAs corresponding to the 68 kd neurofilament protein (NF68) and the Thy-1 glycoprotein within 24 h, with maximal effects of some 90- and 45-fold stimulation (relative to beta-actin mRNA) being observed after 12 and 4 days of treatment, respectively. In addition, transcriptional run-off analyses using isolated nuclei showed that NGF treatment resulted directly in 8- and 4-fold increases in the rate of NF68 and Thy-1 gene transcription. These gene activation events were independent of overt morphological differentiation of PC12 cells occurring both under conditions permissive and non-permissive for neurite outgrowth, and once established the new molecular phenotype was dependent upon the continued presence of NGF. This is the first ...
TY - JOUR. T1 - Cryptic Amyloidogenic Elements in the 3′ UTRs of Neurofilament Genes Trigger Axonal Neuropathy. AU - Rebelo, Adriana P.. AU - Abrams, Alexander J.. AU - Cottenie, Ellen. AU - Horga, Alejandro. AU - Gonzalez, Michael. AU - Bis, Dana M.. AU - Sanchez-Mejias, Avencia. AU - Pinto, Milena. AU - Buglo, Elena. AU - Markel, Kasey. AU - Prince, Jeffrey. AU - Laura, Matilde. AU - Houlden, Henry. AU - Blake, Julian. AU - Woodward, Cathy. AU - Sweeney, Mary G.. AU - Holton, Janice L.. AU - Hanna, Michael. AU - Dallman, Julia E.. AU - Auer-Grumbach, Michaela. AU - Reilly, Mary M.. AU - Zuchner, Stephan. N1 - Funding Information: We deeply appreciate the commitment of the families who participated in this study. This work was supported by the NIH (R01NS075764, U54NS065712, and U54NS092091 to S.Z.), the Charcot-Marie-Tooth Association, the Austrian Science Fund (FWF P23223-B19 and P27634FW), and the Muscular Dystrophy Association. We also thank the Inherited Neuropathy Consortium for advice ...
Our quantitative immunoblot data showed a significant increase of NF-H, NF-M, and NF-L by 3 wk of age in the mutant DRGs, and thus in sensory neuron cell bodies. The simplest explanation is that these NF subunits were synthesized at normal rates but were moved out of the cell bodies at reduced rates. Overall, the levels of these proteins were not significantly changed in the brain, suggesting that the cell body accumulation is not caused by up-regulation of these proteins. Elevation of NF subunit levels in the DRG was not accompanied by obvious reductions in the sciatic nerve. This behavior is as expected based on two independent lines of evidence. First, the onset of the apparent deficit in transport is observed at 3 wk of age, the age at which substantial NF deposition and radial growth in axonal caliber normally begin. Only a subset of axons in mutants examined at this time have detectable caliber deficits (∼250/3,500 total axons in the sciatic nerve). Second, although Cre-mediated excision ...
Riluzole is the only drug approved for the treatment of amyotrophic lateral sclerosis (ALS) but its precise mode of action is not properly understood. Damage to axonal transport of neurofilaments is believed to be part of the pathogenic mechanism in ALS and this has been linked to defective glutamate handling and increased phosphorylation of neurofilament side-arm domains. Here, we show that riluzole protects against glutamate-induced slowing of neurofilament transport. Protection is associated with decreased neurofilament side-arm phosphorylation and inhibition of the activities of two neurofilament kinases, ERK and p38 that are activated in ALS. Thus, the anti-glutamatergic properties of riluzole include protection against glutamate-induced changes to neurofilament phosphorylation and transport. ...
The overall objective of this study was to investigate the role that the small heat shock protein Hsp27 plays in neurite initiation and growth, through its regulation of the neuronal cytoskeleton. The present investigation was carried out using adult rat dorsal root ganglion (DRG) neurons to study the behavior of neurite initiation and extension. -- The extracellular environment triggers the production and extension of a neurite via extracellular cues. To trigger morphological changes in the neuron these cues must be transformed into signals that converge on the cytoskeleton. The cytoskeletal components, such as actin, tubulin and neurofilament light chain (NF-L), can be modified though interaction with other cellular proteins, including Hsp27. Hsp27 is regulated through intracellular signaling cascades, and has been demonstrated to interact with cytoskeletal components. This positioning of Hsp27 as a possible transducer of extracellular signals to the cytoskeleton formed the basis for my ...
Looking for online definition of neurofilament in the Medical Dictionary? neurofilament explanation free. What is neurofilament? Meaning of neurofilament medical term. What does neurofilament mean?
The innervation of the vibrissal follicle sinus complexes (FSCs) in the mystacial pad of the rat was examined by lectin binding histofluorescence with the B subunit of Griffonia simplicifolia (GSA) and by immunofluorescence with a wide variety of antibodies for neuronal related structural proteins, enzymes, and peptides. Only anti-protein gene product 9.5 labeled all sets of innervation. Several types of mechanoreceptors were distributed to specific different targets by medium to large caliber myelinated axons. All were positive for 200 kDa neurofilament subunit, peripherin, and carbonic anhydrase. Their endings expressed synaptophysin. Labeling for the 160 kDa neurofilament subunit, calbindin, and parvalbumin varied. Anti-Schwann cell protein S100 was completely co-extensive with the axons, terminal arbors, and endings of the mechanoreceptor afferents including Merkel innervation. At least 15 different sets of unmyelinated innervation were evident based upon distribution and labeling ...
We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page.. Google Safari Microsoft Edge Firefox. Click here to login. ...
The six types of IFs (types I-VI) are shown. To simplify the schematic, not all epithelial and nonepithelial tissues are displayed. Different keratin pairs are found primarily in unique epithelial cell types in a differentiation state-selective and/or cell type-specific distribution (e.g., K4/K13 in the esophagus, K20 in suprabasal but not in basal crypt enterocytes). The complexity of IF expression in tissues is exemplified in the intestine, in which epithelial cells express different compliments of simple epithelial keratins, the vasculature and other resident mesenchymal cells express vimentin, the smooth muscle layer expresses desmin, and neural elements of the enteric nervous system express neurofilaments. Numbers in parentheses indicate the type of IF. Bfsp1, beaded filament structural protein 1 (previously known as CP115 and filensin); Bfsp2 was previously known as CP49 and phakinin. NFH, high-molecular-weight neurofilament subunit; NFL, low-molecular-weight neurofilament subunit; NFM, ...
Alzheimers disease (AD) and vascular dementia (VaD) are intertwined by mixed dementia (MD) harboring varying degrees of AD pathology in combination with cerebrovascular disease. The aim was to assess whether there is a difference in the cerebrospinal fluid (CSF) profile, of selected proteins, between patients with VaD and MD with subcortical vascular disease (SVD), AD, and healthy controls that could contribute in the separation of the groups. The study included 30 controls, 26 SVD patients (9 VaD and 17 MD) and 30 AD patients. The protein panel included total tau (T-tau), hyperphosphorylated tau 181 (P-tau181), amyloid β 1-42 (Aβ1-42), neurofilament light (NF-L), myelin basic protein (MBP), heart fatty acid binding protein (H-FABP), matrix metalloproteinases (MMP-1, -2, -3, -9, and -10), and tissue inhibitors of metalloproteinases (TIMP-1 and -2). Immunochemical methods were utilized for quantification of the proteins in CSF and data analysis was performed with a multivariate discriminant ...
Huntingtons disease (HD) is an autosomal dominant neurodegenerative disorder, caused by a CAG-repeat expansion in the HTT-gene. Today there are no disease-modifying therapies (DMTs), but several promising clinical trials are underway, including therapies that reduce mutant huntingtin expression.. Reliable biomarkers could empower such trials and guide the timing for initiation of future DMTs.. Neurofilament light (NFL) and tau, which are cerebrospinal fluid markers of neuronal death, have been implicated as markers of disease progression. Increased levels of the inflammatory marker YKL-40 have also been reported in HD.. The aim was to validate and compare the above biomarker candidates by targeted analyses, while explorative liquid chromatography-mass spectrometry (LC-MS) was used to identify new candidates. Clinically well-characterized HD patients, premanifest gene expansion carriers (pGECs), and controls were enrolled from Uppsala University Hospital in Sweden.. In contrast to tau, NFL ...
Objective To investigate the function of homocysteine in neuronal damage in HIV an infection. in both treated and untreated HIV. While this scholarly research struggles to verify a causal hyperlink, homocysteine and useful B12/folate deficiency may actually are likely involved in neural damage in HIV-infected people. 717907-75-0 Introduction Before the breakthrough of effective antiretroviral therapy (Artwork), HIV-infected people had in regards to a 30% general threat of developing HIV-related dementia (HAD) [1]. Today differs However the picture, HIV diagnosis is normally somewhat still connected with HIV-associated neurocognitive disorders (Hands). Symptoms of Hands are normal in untreated HIV-positive people and reported by people on suppressive Artwork [2] frequently. Recent studies have got noted increased degrees of cerebrospinal liquid (CSF) neurofilament light proteins (NFL) in HIV-infected people, which is normally interpreted as an indicator of ongoing neuronal damage. Elevated ...
Rare autosomal recessive disorder of INTERMEDIATE FILAMENT PROTEINS. The disease is caused by mutations in the gene that codes gigaxonin protein. The mutations result in disorganization of axonal NEUROFILAMENT PROTEINS, formation of the characteristic giant axons, and progressive neuropathy. The clinical features of the disease include early-onset progressive peripheral motor and sensory neuropathies often associated with central nervous system involvement (INTELLECTUAL DISABILITY, seizures, DYSMETRIA, and CONGENITAL NYSTAGMUS ...
Christophe participated to the Emerging Concepts in the Neuronal Cytoskeleton meeting in Villarica, Chile. He was the first speaker of the meeting in the Super-resolution microscopy of the neuronal cytoskeleton, straight after three flights and 30 hours of travel! He presented new results on the visualization of axonal actin rings using super-resolution and electron microscopy (see the preprint here). The meeting was excellent with a stunning location by the lake, lots of amazing talks and interesting discussions. Read more on Twitter.. ...
Neurofilament (H+L) (Neuronal Marker) Antibody, Mouse Monoclonal Antibody [Clone NF421 + NFL/736 ] validated in IHC, IF, FC (AH12982-7), Abgent
Amyloid-β plaque accumulation in Alzheimers disease (AD) is associated with dystrophic neurite formation and synapse loss in principal neurons, but interneuron pathology is less clearly characterised. We compared the responses of neuronal processes immunoreactive for either neurofilament triplet (NF+) or calretinin (CR+) to fibrillar amyloid (Aβ) plaques in human end-stage and preclinical AD, as well as in APP/PS1 and Tg2576 transgenic mouse AD models. Neurites traversing the Aβ plaque core, edge or periphery, defined as 50, 100 and 150% of the plaque diameter respectively, in human AD and transgenic mouse tissue were compared to age-matched human and wild-type mouse controls. The proportion of NF+ neurites exhibiting dystrophic morphology (DNs) was significantly larger than the proportion of dystrophic CR+ neurites in both human AD and transgenic mice (p
Iam currently building a outfit based on that of a male southerner who lived in a city that was ransacked by the union army but is now a refugee (a wounded veteran of a earlier war) and was wondering what sort of sidearm would be appropriate.
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I can control what I consume and how I treat and move my body when it is up for exercise, but the rest of it is just riding the waves of whatever is happening on a given day. My schedule is only so much my own with the every other week chemo sessions. Any plans made need to be recognized as changeable. And so I awake each day with ideas for my day, but not necessarily the ability to follow through. I must be accepting of simply flowing like water around and through the days. A sense of control must give way to self-kindness and allowance. These are the subjects of countless platitudes and memes that swirl around the internet. Ideas that we all love the sensibility of, but rarely have the fortitude to put into action. And even in a situation such as mine, with optimism and hope as my sidearms, it is hard to stay on the sunny side of the street all the time. ...
NF-L antibody [1H3] (neurofilament, light polypeptide) for ELISA, FACS, ICC/IF, IHC-P, WB. Anti-NF-L mAb (GTX60541) is tested in Human samples. 100% Ab-Assurance.
Which of the following compounds has the greatest molar solubility? A) AgBr, Ksp= 5.4 x 10^13 B) Ba3(PO4)2, Ksp = 3.0 x 10^-23 C) Al(OH)3, Ksp = 1.9 x 10^-33 D) MgF2, Ksp = 7.4 x 10^-11 E) Pb(OH)2, Ksp = 1.2 x 10^-15 I know the ...
training camps open this week. fuck yeah. Why the NFL is better than MLB: if its late in the season and your team sucks, you can still go to an NFL game and have a good time. I have lots of experience with this. Why the NFL is better than the NBA: they play defense in the NFL. Why the NFL is better than the NHL: fewer canadians. Lots fewer. Why the NFL is better than NCAA football: college ball is played in places like Tuscaloosa and Knoxville and South Bend. College ball
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Colin Kaepernick could be one of the greatest quarterbacks ever. I love his skill set. He throws with accuracy. And in todays NFL, you have to have mobility. Hes got all those…. ...
Its been a month since the suspension of live sports, so all eyes are on how the NFL will conduct the draft & execute fan engagement while we #StayAtHome.
The draft is still quite a ways off, and March free agency will arrive first, but much of the NFL worlds focus has already turned to the April 25-27 event. Its a time of renewed hope for bottom-feeders and Super Bowl contenders alike...
We discuss the hilarious moment when Trevon Diggs son confuses Dak Prescott for Patrick Mahomes. PLUS we play, Real Names, Real NFL Players?... Stay tun
The NFL combine wasnt friendly to all prospects hoping to be selected early on in the 2013 draft, and as a result, their respective pro days will be vital in order to improve their declining draft stock...
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I mean, if he wasnt completely cleared right now, and his condition were currently keeping him from playing/participating in the combine/etc. that would be an enormous red flag. Thats the kind of thing that would get him entirely crossed off a lot of boards. That would indicate that his condition is much worse/more advanced than i think most people were led to believe ...
Tebow will no doubt remain a cultural touchstone. His camp invite alone inspired huge reactions that were both positive and negative. His getting cut will no doubt do the same.
The protein was originally purified from rat optic nerve and spinal cord. The protein copurifies with other neurofilament ... As development continues into neurons the neurofilament triplet proteins (NF-L: neurofilament low molecular mass, NF-M: ... along with the neurofilament triplet proteins. They are expressed in a relatively fixed stoichiometric ratio to neurofilaments ... α-internexin is functionally interdependent with the neurofilament triplet proteins. If one genetically deletes NF-M and/or NF- ...
Neurofilament medium polypeptide (NF-M) is a protein that in humans is encoded by the NEFM gene. Neurofilaments are type IV ... This gene encodes the medium neurofilament protein. This protein is commonly used as a biomarker of neuronal damage. GRCh38: ... "Cytoplasmic O-GlcNAc modification of the head domain and the KSP repeat motif of the neurofilament protein neurofilament-H". J ... 1987). "The human mid-size neurofilament subunit: a repeated protein sequence and the relationship of its gene to the ...
"PKN associates and phosphorylates the head-rod domain of neurofilament protein". J. Biol. Chem. 271 (16): 9816-22. doi:10.1074/ ... Serine/threonine-protein kinase N1 is an enzyme that in humans is encoded by the PKN1 gene. The protein encoded by this gene ... "PKN associates and phosphorylates the head-rod domain of neurofilament protein". J. Biol. Chem. 271 (16): 9816-22. doi:10.1074/ ... "Analysis of RhoA-binding proteins reveals an interaction domain conserved in heterotrimeric G protein beta subunits and the ...
... , also known as neurofilament light chain, is a neurofilament protein that in humans is encoded ... notably the tau and neurofilament light chain proteins. Xu Z, Henderson RD, David M, McCombe PA (2016). "Neurofilaments as ... Neurofilament light polypeptide has been shown to interact with: MAP2, Protein kinase N1, and TSC1. GRCh38: Ensembl release 89 ... Chin SS, Liem RK (December 1989). "Expression of rat neurofilament proteins NF-L and NF-M in transfected non-neuronal cells". ...
"TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein". Molecular and Cellular Neurosciences. 35 (2 ... TAR DNA-binding protein 43 (TDP-43, transactive response DNA binding protein 43 kDa) is a protein that in humans is encoded by ... The entire protein devoid of large solubilising tags has been purified. The full-length protein is a dimer. The dimer is formed ... March 2001). "Toward a catalog of human genes and proteins: sequencing and analysis of 500 novel complete protein coding human ...
For example, there is an increase in phosphorylated neurofilament proteins and cytoskeletal components, tubulin and actin, in ... Goldstein, ME; Cooper, HS; Bruce, J; Carden, MJ; Lee, VM; Schlaepfer, WW (1987). "Phosphorylation of neurofilament proteins and ... The increase in protein can be explained by the increase in cytoskeleton size. Changes in the cell body cytoskeleton seem to be ... Also both seem to be mechanically related to a disruption of the delivery of neurofilament to the axon due to a decreased ...
... negative for neurofilament proteins). There is also considerable clinical interest in the use of neurofilament proteins as ... neurofilament proteins are released into the blood or cerebrospinal fluid. Immunoassays of neurofilament proteins in ... mammalian neurofilaments were originally thought to be composed of just three proteins called neurofilament protein L (low ... The proteins that form neurofilaments are members of the intermediate filament protein family, which is divided into six types ...
Wild's research since 2017 has focused on the potential of neurofilament light and mutant huntingtin protein as biomarkers for ... "Evaluation of mutant huntingtin and neurofilament proteins as potential markers in Huntington's disease". Science Translational ... In 2015 he published the first successful detection and quantification of mutant huntingtin protein (the known cause of ... for the finding that neurofilament light in blood can predict onset and progression of HD). 2018 Elected Fellow of the Royal ...
"Peptidyl-Prolyl Isomerase 1 Regulates Protein Phosphatase 2A-Mediated Topographic Phosphorylation of Neurofilament Proteins". ... The enzyme binds to a subset of proteins and thus plays a role as a post phosphorylation control in regulating protein function ... Pin is a small protein at 18 kDa and does not have a nuclear localization or export signal. However, 2009, Lufei et al. ... "Entrez Gene: PIN1 Protein (peptidylprolyl cis/trans isomerase) NIMA-interacting 1". da Costa, Kauê Santana; Galúcio, João ...
"Effect of Guilingji on expression of neurofilament protein in cerebral cortex and corpus striatum". Chinese Journal of Anatomy ...
Frappier T, Regnouf F, Pradel LA (1988). "Binding of brain spectrin to the 70-kDa neurofilament subunit protein". Eur. J. ... Secondly, another insert of 20 amino acids in the 10th spectrin repeat, termed SH3i+, contains protein kinase A and protein ... Herrmann H, Wiche G (1987). "Plectin and IFAP-300K are homologous proteins binding to microtubule-associated proteins 1 and 2 ... Ankyrin repeats of the multidomain Shank protein family interact with the cytoskeletal protein alpha-fodrin". J. Biol. Chem. ...
This includes synaptophysin, neurofilament protein, and CRX, a specific pineal or retinal marker, positive staining. Initial ...
In contrast, structural proteins such as tubulin and neurofilament subunits are transported at lower rates. Proteins that are ... Microtubule Neurofilament Tubulin Microtubule associated protein Neuronal migration "Medical Definition of NEUROTUBULE". www. ... Microtubule-associated proteins (MAPs) are proteins that interact with microtubules by binding to their tubulin subunits and ... soluble protein. The speed of transport depends on the types of cargo to be transported. Neurotrophins, a family of proteins ...
"Analysis of epitopes shared by Hirano bodies and neurofilament proteins in normal and Alzheimer's disease hippocampus". Lab. ... It was observed that Hirano bodies are a specific site of a C-terminal fragment of β-amyloid precursor proteins. University of ... More specifically the actin and actin binding proteins seen in Hirano bodies are a significant feature of an Alzheimer's ... Hirano bodies are intracellular aggregates of actin and actin-associated proteins first observed in neurons (nerve cells) by ...
Neurofilament, heavy polypeptide (NEFH) is a protein that in humans is encoded by the NEFH gene. It is the gene for a heavy ... protein subunit that is combined with medium and light subunits to make neurofilaments, which form the framework for nerve ...
"Characterization of the human superior olivary complex by calcium binding proteins and neurofilament H (SMI-32)". The Journal ... "Characterization of the rhesus monkey superior olivary complex by calcium binding proteins and synaptophysin". Journal of ...
A preliminary study about neurofilament light chain and tau protein levels in psoriasis: Correlation with disease severity. ... "A preliminary study about neurofilament light chain and tau protein levels in psoriasis: Correlation with disease severity." ... Okan and his team reported their findings that psoriasis patients had unusually high levels of neurofilament and Tau protein, ...
... proteins co-aggregate with spheroid neurofilaments in amyotrophic lateral sclerosis". Journal of Neuropathology and ... Microtubule-associated protein 6 (MAP6) or stable tubule-only polypeptide (STOP or STOP protein) is a protein that in humans is ... A MAP6-related protein, TbSAXO, has been discovered in Trypanosoma brucei. The domains of the protein responsible for ... This gene encodes a microtubule-associated protein (MAP). The encoded protein is a calmodulin-binding and calmodulin-regulated ...
... and these cells form interconnected axon networks and express tetanus toxin receptors and neurofilament proteins. By 10-14 days ... of NTERA-2 clonal human embryonal carcinoma cells into neurons involves the induction of all three neurofilament proteins". J ... as well as microtubule-associated proteins expressed in human neuroepithelium. NTERA-2 cells also accumulate cytoplasmic ...
Other molecules that can be degraded by calpains are microtubule subunits, microtubule-associated proteins, and neurofilaments ... as well as tau protein and amyloid precursor protein (APP) deposition. Lesions typically are found in the white matter of ... One of the proteins activated by the presence of calcium in the cell is calpain, a Ca2+-dependent non-lysosomal protease. About ... "Topography of axonal injury as defined by amyloid precursor protein and the sector scoring method in mild and severe closed ...
... microtubule-associated proteins, and neurofilaments. It may also damage ion channels, other enzymes, cell adhesion molecules, ... to recognize its common properties with two well-known proteins at the time, the calcium-regulated signalling protein, ... A calpain (/ˈkælpeɪn/; EC 3.4.22.52, EC 3.4.22.53) is a protein belonging to the family of calcium-dependent, non-lysosomal ... Amongst protein substrates, tertiary structure elements rather than primary amino acid sequences are likely responsible for ...
"Myotubularin-related 2 protein phosphatase and neurofilament light chain protein, both mutated in CMT neuropathies, interact in ... The protein also contains a GRAM domain. Mutations in this gene are a cause of Charcot-Marie-Tooth disease type 4B, an ... The complete sequences of 100 new cDNA clones from brain which code for large proteins in vitro". DNA Res. 6 (3): 197-205. doi: ... Myotubularin-related protein 2 also known as phosphatidylinositol-3,5-bisphosphate 3-phosphatase or phosphatidylinositol-3- ...
A Lewy body is composed of the protein alpha-synuclein associated with other proteins, such as ubiquitin, neurofilament protein ... Tau proteins may also be present, and Lewy bodies may occasionally be surrounded by neurofibrillary tangles. Lewy bodies and ... When misfolded proteins aggregate, or clump together, many diseases are more likely to develop, including those that are ... He was the first doctor to notice some unusual proteins in the brain, comparing them to earlier findings by Gonzalo Rodríguez ...
Several neuronal markers such as neurofilament proteins, HNK-1 antigen and tetanus toxin binding sites are expressed at highest ... "Bone morphogenetic proteins induce cardiomyocyte differentiation through the mitogen-activated protein kinase kinase kinase ... After 10 days of exposure, astroglial cells can be detected using glial fibrillary acidic protein (GFAP), which is a specific ... The main affected signaling pathway, bone morphogenetic proteins (BMPs) pathway is the most strongly studied signaling in P19 ...
Studies of extracted proteins suggest that this anemone's neurons contain neurofilament-like proteins that are molecularly ...
Finally, a loss of vesicular monoamine transporters, neurofilament proteins, and other morphological changes appear to indicate ...
... but it can also heteropolymerize with neurofilaments in several neuronal types. This protein in humans is encoded by the PRPH ... Its size, structure, and sequence/location of protein motifs is similar to other type III intermediate filament proteins such ... with other type III proteins or the light neurofilament subunit (NF-L) to form intermediate filament networks. Type III ... glial fibrillary acidic protein, and desmin. All intermediate filament proteins share a common secondary structure consisting ...
Neurofilament light chain is a protein that is important in the growth and branching of neurons-cells found in the brain. In ... Atrophy of any tissue means a decrement in the size of the cell, which can be due to progressive loss of cytoplasmic proteins. ... Other biomarkers like Ng - a protein important in long-term potentiation and memory - have been tracked for their associations ... One study took advantage of biomarkers, namely one called neurofilament light chain (NFL), in patients with Alzheimer's Disease ...
Seven genes encode for the heavy (NF-H), medium (NF-M) and light neurofilament (NF-L) proteins, nestin and α-internexin in ... Nestin is a protein that in humans is encoded by the NES gene. Nestin (acronym for neuroepithelial stem cell protein) is a type ... neurofilaments and glial fibrillary acidic protein (GFAP). Nestin expression is reinduced in the adult during pathological ... These intermediate filament proteins are expressed mostly in nerve cells where they are implicated in the radial growth of the ...
... and neurofilament protein. Some medulloblastomas may also display other forms of differentiation as demonstrated by the ... presence of the astrocytic marker glial fibrillary acidic protein. Skeletal muscle and melanocytic differentiation are ...
... neurofilament protein) → - CD56/N-CAM (neural cell adhesion molecule) → ++ S100 → ++ Transthyretin → - Vimentin → +++ Desmin ... These cells have proteins that make up the characteristics of the tumor. These proteins arise from blood vessels, nerve cells ... The cytoplasmic and often nuclear expression of S100 protein is present in nearly all tumor cells, and vimentin typically ... glial fibrillary acidic protein) → + Synaptophysin → - Chromogranin → - NSE (neuron-specific enolase) → ++ NFP ( ...
... s express chemosensory proteins, like TRPM5 and α-gustducin. These proteins indicate that neighbouring neurons can ... Tuft cells can be identified by staining for cytokeratin 18, neurofilaments, actin filaments, acetylated tubulin, and DCLK1 to ...
... nerve tissue protein - nerve tissue protein S 100 - nervous system - neurobiology - neurofilament protein - neurokinin A - ... protein - protein biosynthesis - Protein Data Bank - protein design - protein expression - protein folding - protein isoform - ... protein P16 - protein P34cdc2 - protein precursor - protein structure prediction - protein subunit - protein synthesis - ... proto-oncogene protein C-kit - proto-oncogene proteins c-abl - proto-oncogene proteins c-bcl-2 - Proto-oncogene proteins c-fos ...
In the CNS the major myelin protein is proteolipid protein, and in the PNS it is myelin basic protein. Nodes of Ranvier (also ... and neurofilaments, in both directions between the axon and its terminals and the cell body. Outgoing anterograde transport ... The Nissl bodies that produce the neuronal proteins are absent in the axonal region. Proteins needed for the growth of the axon ... There are many forms of kinesin and dynein motor proteins, and each is thought to carry a different cargo. The studies on ...
Immunofluorescent antibody staining against neurofilament (green) and Ki-67 (red) in a mouse embryo 12.5 days after ... Protein Ki-67 in human MCF-7 cells Ki-67 protein (red), tubulin (green) and DNA (blue) in HeLa cells. Dividing cells show ... whereas in mitosis most of the protein is relocated to the surface of the chromosomes. Ki-67 protein is present during all ... The Ki-67 protein (also known as MKI67) is a cellular marker for proliferation, and can be used in immunohistochemistry. It is ...
... a protein (enzyme) that catalyzes (increases the rates of) chemical reactions Organophosphate-induced delayed neuropathy, a ... a rare neurological disorder that causes disorganization of neurofilaments Hereditary neuropathy with liability to pressure ...
The presence of an aromatic residue, usually a tryptophan, in the CaM-binding region of Ca2+-CaM-regulated proteins may also be ... PDE1A2 has a potential role in neurodegenerative diseases, including: Parkinson's disease Axonal neurofilament degradation ... Thevelein JM, de Winde JH (September 1999). "Novel sensing mechanisms and targets for the cAMP-protein kinase A pathway in the ... Phosphorylation of PDE1A1 and PDE1A2 by protein kinase A and of PDE1B1 by CaM Kinase II decreases their sensitivity to ...
The abnormal distribution of gap junction proteins such as GJA1 (also known as Connexin 43), and GJA5 (Connexin 40) causes non- ... "Serum Neurofilament Light Chain in Patients With Atrial Fibrillation". Journal of the American Heart Association. 11 (14): ... and Alzheimer disease and with elevated levels of neurofilament light chain in blood, a biomarker indicating neuroaxonal injury ... "Rare truncating variants in the sarcomeric protein titin associate with familial and early-onset atrial fibrillation". Nature ...
... monosomy 7 Mouse orthologue of ARX MRP4 Msi-1 Musashi Musashi-1 Mutant BCRP nestin neurofilament microtubule-associated protein ... Below is a list of genes/protein products that can be used to identify various types of stem cells, or functional assays that ... Stahl J, Wobus AM, Ihrig S, Lutsch G, Bielka H (September 1992). "The small heat shock protein hsp25 is accumulated in P19 ... Stem cell markers are genes and their protein products used by scientists to isolate and identify stem cells. Stem cells can ...
Although the protein created localizes within the nucleus and is barely detectable in axons, studies suggest that its ... The depolymerization of microtubules occurs and is soon followed by degradation of the neurofilaments and other cytoskeleton ... The SARM1 protein has four domains, a mitochondrial localization signal, an auto-inhibitory N-terminus region consisting of ... The protective effect of the WldS protein has been shown to be due to the NMNAT1 region's NAD+ synthesizing active site. ...
She had heard that Down syndrome was a disease of the neurofilaments; this turned out not to be the cause, but it was noted ... This was the first time a researcher demonstrated that a single amino acid exchange in a protein can cause a disease or ... At Rockefeller, he worked with Moses Kunitz on crystallising proteins. While at Yale, he studied peptide chemistry with Joseph ... studying protein chemistry. In 1956, Ingram, John A. Hunt, and Antony O. W. Stretton determined that the change in the ...
Some cases of CIDP are reported to be produced by auto-antibodies against several neurofascin proteins. These proteins are ... February 2019). "Prognostic value of serum neurofilaments in patients with clinically isolated syndromes". Neurology. 92 (7): ... contactin associated protein 1 (CASPR1) and gliomedin. All of them nodal and paranodal proteins. Several anti-TNF drugs like ... in MS-like and ADEM-like Proteomic analysis have shown that two proteins, Secretogranin II and Protein 7B2, in CSF can be used ...
... the roles of proteins showing loss of nerve tissue such as neurofilaments, tau, and N-acetylaspartate are under investigation. ... October 2018). "Neurofilaments as biomarkers in neurological disorders" (PDF). Nature Reviews. Neurology. 14 (10): 577-589. doi ... Petzold A (June 2005). "Neurofilament phosphoforms: surrogate markers for axonal injury, degeneration and loss" (PDF). Journal ... and more activation of cytokines and other destructive proteins. Inflammation can potentially reduce transmission of ...
One of these is the phosphorylated, axonal form of the major neurofilament protein heavy chain protein which has the HGNC name ... Another class of antibody to neurofilament NF-L was shown to bind only neurofilaments in healthy neurons and their processes ... Two further studies describe novel EnCor assays for UCHL1 and alpha-synuclein, two major brain proteins implicated in the ... Some have become useful for diagnostic histopathology and for monitoring the levels of protein biomarkers, of research and ...
The GABAA beta-2 subunit is a protein that in humans is encoded by the GABRB2 gene. It combines with other subunits to form the ... "Genome-wide association study identifies two loci influencing plasma neurofilament light levels". BMC Medical Genomics. 11 (1 ... GABRB2+protein,+human at the US National Library of Medicine Medical Subject Headings (MeSH) Overview of all the structural ... GABRB2 includes a consensus sequence for a calmodulin-dependent protein kinase II within exon 10 which is only expressed by β2L ...
Tuberous sclerosis 1 (TSC1), also known as hamartin, is a protein that in humans is encoded by the TSC1 gene. TSC1 functions as ... "The TSC1 tumor suppressor hamartin interacts with neurofilament-L and possibly functions as a novel integrator of the neuronal ... Two proteins involved in tuberous sclerosis". The Journal of Biological Chemistry. 276 (24): 21017-21. doi:10.1074/jbc. ... TSC1, TSC2 and TBC1D7 is a multi-protein complex also known as the TSC complex. This complex negatively regulates mTORC1 ...
Several extracellular matrix proteins are enriched at nodes of Ranvier, including tenascin-R, Bral-1, and proteoglycan NG2, as ... The decreased axon size reflects a higher packing density of neurofilaments in this region, which are less heavily ... All of these proteins, including ankyrin, are enriched in the initial segment of axons which suggests a functional relationship ... The proteins in these excitable domains of neuron when injured may result in cognitive disorders and various neuropathic ...
Syncoilin is an atypical type III IF protein. Alpha-internexin Neurofilaments - the type IV family of intermediate filaments ... There are four proteins classed as type III intermediate filament proteins, which may form homo- or heteropolymeric proteins. ... Animal IFs are subcategorized into six types based on similarities in amino acid sequence and protein structure: These proteins ... Quinlan R, Hutchison C, Lane B (1995). "Intermediate filament proteins". Protein Profile. 2 (8): 795-952. PMID 8771189. Helfand ...
... enzymes regulating protein expression, and proteins related to neuronal/synaptic function, such as neurofilament light protein ... Therefore, there is a focus on expanding the number of measurable biomarkers by including proteins related to the neuronal ...
Cite this: Serum Neurofilament Light Protein Highly Anticipated Tool for MS - Medscape - Mar 05, 2019. ... UPDATED March 11, 2019 // DALLAS - Mounting evidence on the reliability of blood-derived neurofilament light protein (NfL) in ... "The Neurologists C-Reactive Protein" In a separate talk at the meeting, Gavin Giovannoni, PhD, professor of neurology at the ... "I think our C-reactive protein is coming and its going to be in the form of peripheral blood NfL," he said, in reference to an ...
Here, we describe neurofilament light chain (NFL), a marker for neuroaxonal damage, as a translational surrogate marker for ...
Diagnostic Value of Cerebrospinal Fluid Neurofilament Light Protein in Neurology Bridel C., van Wieringen WN., Zetterberg H., ...
Cite this: Serum Neurofilament Light Protein Highly Anticipated Tool for MS - Medscape - Mar 05, 2019. ... UPDATED March 11, 2019 // DALLAS - Mounting evidence on the reliability of blood-derived neurofilament light protein (NfL) in ... "The Neurologists C-Reactive Protein" In a separate talk at the meeting, Gavin Giovannoni, PhD, professor of neurology at the ... "I think our C-reactive protein is coming and its going to be in the form of peripheral blood NfL," he said, in reference to an ...
These findings support the neurofilament protein as a biomarker for disease activity ... ... A protein called neurofilament light chain (NFL), which supports nerve fibers, is elevated in adults with neurosarcoidosis - ... Neurofilaments are proteins that provide structural support for nerve fibers, called axons. One component of neurofilaments is ... A protein called neurofilament light chain (NFL), which supports nerve fibers, is elevated in adults with neurosarcoidosis - ...
Neurofilament protein subunits are potential cerebrospinal fluid (CSF) biomarkers for disease progression in MS. We argue that ... Neurofilament protein subunits are potential cerebrospinal fluid (CSF) biomarkers for disease progression in MS. We argue that ... The neurofilament intermediate subunit has not yet been studied. Recent studies showing higher neurofilament light or heavy ... Neurofilaments as biomarkers in multiple sclerosis Mult Scler. 2012 May;18(5):552-6. doi: 10.1177/1352458512443092. Epub 2012 ...
keywords = "Amino acid composition, Neurofilament triplet proteins, Neurofilaments",. author = "Hogue‐Angeletti, {R. A.} and Wu ... Preparative Separation and Amino Acid Composition of Neurofilament Triplet Proteins. R. A. Hogue‐Angeletti, H. ‐L Wu, W. W. ... Solubilized neurofilament triplet proteins of molecular weights approximately 68,000 (P68), 150,000 (P150), and 200,000 (P200) ... Solubilized neurofilament triplet proteins of molecular weights approximately 68,000 (P68), 150,000 (P150), and 200,000 (P200) ...
Plasma neurofilament light chain protein as a predictor of days in delirium and deep sedation, mortality and length of stay in ... Plasma neurofilament light protein (NfL) is a sensitive indicator of neuronal damage. We undertook an exploratory observational ... Plasma neurofilament light chain protein as a predictor of days in delirium and deep sedat ...
Mutations in the small heat-shock protein HSPB1 (HSP27) are responsible for one form of dHMN. In this study, we have analysed ... including neurofilament middle chain subunit (NF-M) and p150 dynactin. These findings suggest a possible pathogenic mechanism ... Microtubule-Associated Proteins, Mitochondria, Motor Neuron Disease, Mutation, Neoplasm Proteins, Neurofilament Proteins, ... A mutation in the small heat-shock protein HSPB1 leading to distal hereditary motor neuronopathy disrupts neurofilament ...
Mutations in the small heat-shock protein HSPB1 (HSP27) are responsible for one form of dHMN. In this study, we have analysed ... including neurofilament middle chain subunit (NF-M) and p150 dynactin. These findings suggest a possible pathogenic mechanism ... Microtubule-Associated Proteins, Mitochondria, Motor Neuron Disease, Mutation, Neoplasm Proteins, Neurofilament Proteins, ... A mutation in the small heat-shock protein HSPB1 leading to distal hereditary motor neuronopathy disrupts neurofilament ...
However, MCC expresses neurofilament proteins not observed in normal Merkel cells. MCC seldom expresses vasoactive intestinal ... 38] Other proteins are the target of experimental vaccines. [39] Further exploration of virus-specific therapy will provide ... Houben R, Adam C, Baeurle A, Hesbacher S, Grimm J, Angermeyer S. An intact retinoblastoma protein-binding site in Merkel cell ... 8] Research into the genome of MCV is expanding and specific proteins are being explored for their clinical utility. For ...
NF-220 protein, Loligo pealei. Known as: neurofilament protein NF 220, Loligo pealei ...
1998) Aggregation of neurofilament and α-synuclein proteins in Lewy bodies. Arch Neurol 55:151-152. ... The ordered aggregation of fibrillogenic proteins into amyloid is most efficient above a critical protein concentration (Harper ... Similarities in the biophysical properties of amyloidogenic proteins suggest that diseases characterized by abnormal protein ... but the universal tendency of the offending proteins to self-aggregate suggests that ordered protein polymerization is ...
CSF neurofilament and glial fibrillary acidic protein in normal pressure hydrocephalus. Mats Tullberg, L Rosengren, E ... Ventricular cerebrospinal fluid neurofilament protein levels decrease in parallel with white matter pathology after shunt ... Dynamics of extracellular matrix proteins in cerebrospinal fluid and serum and their relation to clinical outcome in human ... Lumbar and ventricular CSF concentrations of extracellular matrix proteins before and after shunt surgery in idiopathic normal ...
Background Neurofilaments (Nf) are major structural proteins that occur exclusively in neurons. In spinal cord injury (SCI), ... Serum neurofilament light chain is a biomarker of human spinal cord injury severity and outcome ... Serum neurofilament light chain is a biomarker of human spinal cord injury severity and outcome ...
Neurofilaments (NF) are type IV intermediate filament heteropolymers composed of light, medium, and heavy chains. Detection of ... Neurofilament light is a 68 kD protein. Distribution Cytoskeleton and the cytoplasm. ... Neurofilament Light Polypeptide 68kD, Neurofilament Light Polypeptide, Neurofilament Subunit NF-L, CMT1F, NFL, NEFL, NF-L ... View All Neurofilament L (NF-L) Reagents Request Custom Conjugation Description. Clone. Applications. ...
... and neurofilament light chain (NF-L) were measured at 48h, 14d and 30d post-injury. Fatty acid composition of food, plasma and ... and neurofilament light chain (NF-L) were measured at 48 h, 14 days, and 30 days post-injury. Fatty acid composition of food, ... Plasma levels of Total tau (T-tau), glial fibrillary acidic protein (GFAP), ubiquitin c-terminal hydrolase L1 (UCH-L1) ... Plasma levels of Total tau (T-tau), glial fibrillary acidic protein (GFAP), ubiquitin c-terminal hydrolase L1 (UCH-L1) ...
The discovery of specific genes and proteins associated with AD, and the development of new technologies for the production of ... composed by neurofilaments and hyperphosphorylated tau protein, a microtubule associated polypeptide. These two markers are ... Daigle I, Li C: apl-1, a Caenorhabditis elegans gene encoding a protein related to the human beta-amyloid protein precursor. ... When zebrafish PSEN1 protein expression was driven at high levels in cultured human HEK293 cells, the zebrafish protein ...
... and these proteins can be measured in blood," he says. "One of these proteins is neurofilament light chain (NfL), the focus in ... Korley is the author of a new study that measured levels of a blood protein in patients with diabetes, who had not previously ... "However, these small strokes may result in the release of proteins that are associated with brain cell death, ...
Exposure to CS2 resulted in increased phosphorylation of endogenous MAP-2 and exogenously added neurofilament triplet proteins ... Animal studies; Animals; Proteins; Central nervous system; Inhalation studies; Author Keywords: Carbon disulfide; CaM kinase II ... There also was an observed increase in the autophosphorylation of Ca2+/calmodulin-dependent protein kinase II (CaM kinase II). ... Carbon disulfide inhalation increases Ca2+/calmodulin-dependent kinase phosphorylation of cytoskeletal proteins in the rat ...
... macrophage chemoattractant protein 1 (MCP1) in CSF alone; and neurofilament-Lightchain (NfL) and IL-10, among others, in serum ...
Neurofilament light protein in blood as a potential biomarker of neurodegeneration in Huntingtons disease: a retrospective ... SSSNFL - Serum neurofilament light chain (pg/ml). Variable Name: SSSNFL. SAS Label: Serum neurofilament light chain (pg/ml). ... SSNFLH - Serum neurofilament above detect limit. Variable Name: SSNFLH. SAS Label: Serum neurofilament above detect limit. ... SSNFLL - Serum neurofilament below detect limit. Variable Name: SSNFLL. SAS Label: Serum neurofilament below detect limit. ...
NeuN and neurofilament H (NF-H) staining were performed, and additionally a pS6 antibody was used to demonstrate mTOR pathway ... The Nissl body cannot be seen due to the accumulation of neurofilament proteins (Fig. 3).13 ... NeuN and neurofilament H (NF-H) staining were performed, and additionally a pS6 antibody was used to demonstrate mTOR pathway ... NeuN and neurofilament H (NF-H) staining were performed, and additionally a pS6 antibody was used to demonstrate mTOR pathway ...
Furthermore, the protein level of Bax was decreased whereas the protein levels of Bcl-2 and NF200 were increased after the GTP ... We also studied the cytoskeletal integrity of neurons, by observing the expression of neurofilament protein (NF200) in the ... Furthermore, the protein level of Bax was decreased whereas the protein levels of Bcl-2 and NF200 were increased after the GTP ... Protein concentration was determined with the assay kit (Biotime Biotechnology, China). Proteins were separated ...
... imaging of fluorescently tagged neurofilament proteins has shown that these proteins move in the form of neurofilament polymers ... neurofilaments containing a photoactivatable neurofilament protein are marked by photoactivation of a short segment of axon, ... Koehnle TJ, Brown A (1999) Slow axonal transport of neurofilament protein in cultured neurons. J Cell Biol 144:447-458. doi: ... 1C]. The amount of neurofilament in the central window declines by the sum of the neurofilament fluxes j. a. and j. r. due to ...
Plasma Neurofilament Light and Glial Fibrillary Acidic Protein Levels over 30 Days in a Porcine Model of Traumatic Brain Injury ...
Background: Neurofilament proteins as biomarkers of axonal degeneration have the potential to improve our capacity to predict ... Results and Conclusions: Neurofilament light protein may be more useful as a measure of ongoing neurodegenerative activity in ... Neurofilament Protein as a Potential Biomarker of Axonal Degeneration in Experimental Autoimmune Encephalomyelitis. p. 364. ... Aims: To highlight the possible roles of neurofilament proteins in physiological and pathophysiological processes in the MS. ...
A growing body of evidence suggests that the plasma concentration of the neurofilament light chain (NfL) might be considered a ... Neurofilaments (Nf) consist of three types of protein chains, differing in molecular mass: a light chain (NfL) of 68 kD, an ... Serum neurofilament light chain protein is a measure of disease intensity in frontotemporal dementia. Neurology. 2016;87:1329- ... Neurofilament light chain protein as a marker of neuronal injury: review of its use in HIV-1 infection and reference values for ...
Neurofilament Proteins/blood , tau Proteins/blood , Biomarkers , COVID-19/mortality , Glial Fibrillary Acidic Protein/blood , ... Astrocytes/metabolism , Coronavirus Infections/blood , Glial Fibrillary Acidic Protein/blood , Neurofilament Proteins/blood , ... neurofilament light chain protein (NfL; a marker of intra-axonal neuronal injury) and glial fibrillary acidic protein (GFAp; a ... COVID-19/blood , Neurofilament Proteins/blood , Adult , Aged , Aged, 80 and over , COVID-19/physiopathology , COVID-19/therapy ...
  • Solubilized neurofilament triplet proteins of molecular weights approximately 68,000 (P68), 150,000 (P150), and 200,000 (P200) were purified by preparative electrophoresis, using an LKB 7900 Uniphor apparatus. (elsevier.com)
  • Amino acid compositions of the purified neurofilament triplet proteins are reported and compared. (elsevier.com)
  • Exposure to CS2 resulted in increased phosphorylation of endogenous MAP-2 and exogenously added neurofilament triplet proteins. (cdc.gov)
  • These cells express many properties of motor neurons, including choline acetyltransferase, acetylcholine synthesis, storage and release and neurofilament triplet proteins. (cedarlanelabs.com)
  • They consist of three distinct polypeptides, the neurofilament triplet. (bvsalud.org)
  • 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. (bvsalud.org)
  • Plasma levels of Total tau (T-tau), glial fibrillary acidic protein (GFAP), ubiquitin c-terminal hydrolase L1 (UCH-L1) and neurofilament light chain (NF-L) were measured at 48 h, 14 days, and 30 days post-injury. (frontiersin.org)
  • Plasma Neurofilament Light and Glial Fibrillary Acidic Protein Levels over 30 Days in a Porcine Model of Traumatic Brain Injury Journal of Neurotrauma 2022. (upenn.edu)
  • METHODS: We collected blood from 104 patients infected with SARS-CoV-2 the day of admission to the emergency room and measured blood neurofilament light chair (NfL), glial fibrillary acidic protein (GFAP), ubiquitin carboxy-terminal hydrolase L1 (UCH-L1), and total tau protein levels. (bvsalud.org)
  • Heterogeneity of the glial fibrillary acidic protein in gliosed human brains. (wikidata.org)
  • Glial fibrillary acidic protein from normal and gliosed human brain. (wikidata.org)
  • Furthermore, we performed immunohistochemical studies, including synaptophysin, glial fibrillary acidic protein (GFAP), neurofilament, and Ki-67 to characterize the immunophenotypical patterns of medulloblastoma correlated with morphological variants. (who.int)
  • Neurofilament protein subunits are potential cerebrospinal fluid (CSF) biomarkers for disease progression in MS. We argue that the neurofilament light subunit can reflect acute axonal damage mediated by inflammatory mechanisms and can imply prognostic value for conversion from clinically isolated syndrome (CIS) to definite MS. The neurofilament heavy subunit may rather reflect chronic irreversible damage and has prognostic value for disease progression or disability. (nih.gov)
  • The neurofilament intermediate subunit has not yet been studied. (nih.gov)
  • Recent studies showing higher neurofilament light or heavy subunit levels to be altered upon treatment regimes indicate their potential clinical value in monitoring treatment or side effects. (nih.gov)
  • Mutant (P182L) but not wild-type HSPB1 led to the formation of insoluble intracellular aggregates and to the sequestration in the cytoplasm of selective cellular components, including neurofilament middle chain subunit (NF-M) and p150 dynactin. (ox.ac.uk)
  • Myers MW, Lazzarini RA, Lee VM, Schlaepfer WW, Nelson DL: The human mid-size neurofilament subunit: a repeated protein sequence and the relationship of its gene to the intermediate filament gene family. (t3db.ca)
  • The protein subunit of calf brain neurofilament. (wikidata.org)
  • The study, " Elevated Neurofilament Light Chain in Cerebrospinal Fluid and Plasma Reflect Inflammatory MRI Activity in Neurosarcoidosis ," was published in the journal Brain Sciences . (wijhebbensarcoidose.nl)
  • Diagnostic methods for neurosarcoidosis, such as MRI or CT scans, detect inflammation of the brain and spinal cord, and lab tests can measure elevated immune cells and proteins in the cerebrospinal fluid (CSF) that surrounds the brain and spinal cord. (wijhebbensarcoidose.nl)
  • Cerebrospinal fluid growth-associated protein 43 levels in patients with progressive and stable mild cognitive impairment. (wjgnet.com)
  • This review describes current published findings within the past 3 years in biomarker research in MS, specifically highlighting recent advances in the validation of cerebrospinal fluid biomarkers such as neurofilaments (light and heavy chains), chitinases and chitinase 3-like proteins, soluble surface markers of innate immunity, and oligoclonal immunoglobulin M antibodies. (dovepress.com)
  • We analysed serum neurofilament light chain (NfL) levels in all cases including a subgroup (n = 29) of patients with available onset samples. (bvsalud.org)
  • Serum neurofilament light chain (sNfL) is a specific biomarker of neuronal injury. (bvsalud.org)
  • Biomarkers, for the purposes of this review, are molecular markers detectable in bodily fluids either by immunoassays (enzyme-linked immunoabsorbent assays [ELISAs]) to detect soluble protein biomarkers or quantitative polymerase chain reaction (PCR) to detect circulating miRNAs. (dovepress.com)
  • UPDATED March 11, 2019 // DALLAS - Mounting evidence on the reliability of blood-derived neurofilament light protein (NfL) in predicting disease activity and treatment response in multiple sclerosis (MS) is raising hopes among experts about its utility as an invaluable, easily accessible prognostic tool in the near future. (medscape.com)
  • A general marker of damage to neurons, the NfL protein has been used to measure or monitor the severity of several neurologic diseases, including Alzheimer's disease and multiple sclerosis. (clpmag.com)
  • A protein called neurofilament light chain (NFL), which supports nerve fibers, is elevated in adults with neurosarcoidosis - and appears to increase with more extensive brain inflammation - a study demonstrated. (wijhebbensarcoidose.nl)
  • Analyzing blood samples from patients before, during, and after CAR-T cell therapy, the researchers found that levels of a protein called neurofilament light chain (NfL) are higher in patients who go on to develop neurotoxic complications. (clpmag.com)
  • Neurofilaments are proteins that provide structural support for nerve fibers, called axons. (wijhebbensarcoidose.nl)
  • Neurofilaments are abundant space-filling cytoskeletal polymers that are transported into and along axons. (eneuro.org)
  • Neurofilaments were photoactivated in short segments of myelinated axons in tibial nerves at 2, 4, 8, and 16 weeks of age. (eneuro.org)
  • Neurofilaments are important structural components of axons that are transported from the cell body to the axon tip in a "stop-and-go" manner. (eneuro.org)
  • Here we show that neurofilament transport is bidirectional in mature myelinated axons in vivo , with a significant fraction of the filaments moving backward toward the cell body. (eneuro.org)
  • The fact that axons invest metabolic energy to move neurofilaments backwards as well as forwards in axons is puzzling, and it suggests that neurofilament transport is not simply a mechanism to deliver neurofilaments to axons. (eneuro.org)
  • We speculate that the bidirectional movement of neurofilaments functions to also distribute and organize these polymers in axons, which is a different way of thinking about axonal transport. (eneuro.org)
  • This axonal expansion is driven in part by an accumulation of neurofilaments, which are space-filling cytoskeletal polymers that move into and along axons by the mechanisms of axonal transport ( Hoffman, 1995 ). (eneuro.org)
  • Thus, neurofilaments are an important determinant of one of the basic cable properties of axons. (eneuro.org)
  • Fibrillar proteins from squid axons. (wikidata.org)
  • Type III intermediate filament proteins that assemble into neurofilaments, the major cytoskeletal element in nerve axons and dendrites. (bvsalud.org)
  • A recently described new technique for investigating axon damage depends for its ability on the immunoreactivity of amiloid precursor protein (APP), which has been shown to be more sensitive than silver stains for detecting damaged axons. (ox.ac.uk)
  • In nerve cells, this protein helps to organize a network of molecular threads called neurofilaments that maintain the diameter of specialized extensions called axons. (medlineplus.gov)
  • Studies suggest that the altered protein may be more likely to form clusters (aggregates) and block the transport of substances that are essential for the proper function of nerve axons, leading to the signs and symptoms of distal hereditary motor neuropathy, type II. (medlineplus.gov)
  • A mutation in the small heat-shock protein HSPB1 leading to distal hereditary motor neuronopathy disrupts neurofilament assembly and the axonal transport of specific cellular cargoes. (ox.ac.uk)
  • Studies on cultured nerve cells have shown that axonal neurofilaments move rapidly and intermittently along microtubule tracks in both anterograde and retrograde directions. (eneuro.org)
  • The axonal transport of neurofilament proteins was discovered using radioisotopic pulse-labeling ( Hoffman and Lasek, 1975 ). (eneuro.org)
  • Neurofilaments (Nf) consist of three types of protein chains, differing in molecular mass: a light chain (NfL) of 68 kD, an intermediate chain of 150 kD, and a heavy chain of 190 to 210 kD, and are major components of axonal cytoskeleton [ 6 ]. (biomedcentral.com)
  • Ackerley S, James PA, Kalli A, French S, Davies KE, Talbot K. A mutation in the small heat-shock protein HSPB1 leading to distal hereditary motor neuronopathy disrupts neurofilament assembly and the axonal transport of specific cellular cargoes. (medlineplus.gov)
  • Individually, neurodegeneration in ALS might result from a complex interaction of glutamate excitotoxicity, the genesis of free radicals, SOD1 enzymes, disruption of axonal transport processes through aggregation of neurofilament intracellular accumulates, cytoplasmic protein accumulates, combined with mitochondrial dysfunction. (medacess.com)
  • In addition, they appear to be involved in activities such as cell movement (motility), stabilizing the cell's structural framework (the cytoskeleton), folding and stabilizing newly produced proteins, and repairing damaged proteins. (medlineplus.gov)
  • Here, we describe neurofilament light chain (NFL), a marker for neuroaxonal damage, as a translational surrogate marker for CIPN.METHODS NFL concentrations were measured in an in vitro model of CIPN, exposing induced pluripotent stem cell-derived sensory neurons (iPSC-DSNs) to paclitaxel. (jci.org)
  • Background Neurofilaments (Nf) are major structural proteins that occur exclusively in neurons. (bmj.com)
  • Human stem cell-derived sensory neurons, fluorescently labeled to reveal neurofilament proteins (red and green) and cell nuclei (blue). (nih.gov)
  • 2 Previously, attempts have been made to control the structural polarization of cultured neurons by using several key strategies such as molecular cues of diffusible gradient or substrate-bound chemical/extracellular matrix (ECM) protein patterns, and topographical cues. (cdc.gov)
  • Researchers based at the University of Southern Denmark now wondered if neurofilament light chain could be used as a biomarker in neurosarcoidosis. (wijhebbensarcoidose.nl)
  • Whether neurofilament light chain levels correlated with more extensive inflammation on MRI scans of neurosarcoidosis patients also was investigated. (wijhebbensarcoidose.nl)
  • Plasma neurofilament light chain protein as a predictor of days in delirium and deep sedation, mortality and length of stay in critically ill patients. (bvsalud.org)
  • One of these proteins is neurofilament light chain (NfL), the focus in our study. (dlife.com)
  • A growing body of evidence suggests that the plasma concentration of the neurofilament light chain (NfL) might be considered a plasma biomarker for the screening of neurodegeneration in Alzheimer's disease (AD). (biomedcentral.com)
  • METHODS: In this single centre prospective study, serum levels of neurofilament light chain (sNfL) and glial fibrillar acidic protein (sGFAp) were assessed using SimoaTM assay Neurology 2-Plex B Assay Kit, in 148 hospitalised patients with COVID-19 without clinical neurological manifestations and compared them to 53 patients with interstitial pulmonary fibrosis (IPF) and 108 healthy controls (HCs). (bvsalud.org)
  • Blood will be collected for measurement of other markers of neurodegeneration and oxidative stress, including neurofilament light chain, isoprostanes, and acute phase proteins. (who.int)
  • Cytoskeletal proteins as targets for organophosphorous compound and aliphatic hexacarbo n- induced neurotoxicity. (cdc.gov)
  • Carbon disulfide inhalation increases Ca2+/calmodulin-dependent kinase phosphorylation of cytoskeletal proteins in the rat central nervous system. (cdc.gov)
  • The Ca2+/calmodulin-dependent phosphorylation of neuronal cytoskeletal proteins was studied in brain supernatants prepared from rats exposed via inhalation to 600 or 800 ppm carbon disulfide (CS2) for 14 days. (cdc.gov)
  • Types I, II, and IV intermediate filament proteins form other cytoskeletal elements such as keratins and lamins. (bvsalud.org)
  • Another AD hallmark are the NFT, composed by neurofilaments and hyperphosphorylated tau protein, a microtubule associated polypeptide. (springer.com)
  • HSPB1 gene mutations that cause distal hereditary motor neuropathy, type II change single protein building blocks (amino acids) in heat shock protein beta-1. (medlineplus.gov)
  • Abstract: Intact neurofilaments were isolated from bovine spinal cord white matter, washed by sedimentation in 0.1 m‐NaCl, and extracted with 8 m‐urea. (elsevier.com)
  • Plasma neurofilament light protein (NfL) is a sensitive indicator of neuronal damage. (bvsalud.org)
  • Neurofilaments comprise the axoskeleton and they functionally maintain the neuronal caliber. (biolegend.com)
  • Over the years, such experiments have demonstrated that a pulse of radiolabeled, newly synthesized neurofilament proteins forms a spreading Gaussian wave that propagates slowly in an anterograde direction with a modal velocity ranging from 0.1 to 1 mm/d, depending on neuronal cell type and age ( Brown, 2014 ). (eneuro.org)
  • Neurofilaments usually contain three intermediate filament proteins: L, M, and H which are involved in the maintenance of neuronal caliber. (t3db.ca)
  • Heat shock proteins block signals that lead to programmed cell death. (medlineplus.gov)
  • Heat shock proteins also appear to play a role in the tensing of muscle fibers (muscle contraction). (medlineplus.gov)
  • Mutations of small heat shock proteins and human congenital diseases. (medlineplus.gov)
  • Dierick I, Irobi J, De Jonghe P, Timmerman V. Small heat shock proteins in inherited peripheral neuropathies. (medlineplus.gov)
  • There is no statistical difference in immunophenotypes of synaptophysin, GFAP, or neurofilament between each subtype. (who.int)
  • Neurofilaments (NF) are type IV intermediate filament heteropolymers composed of light, medium, and heavy chains. (biolegend.com)
  • The main constituent of the amyloid deposits is an amphiphilic peptide, derived by proteolysis from a large membrane spanning precursor protein, the amyloid precursor protein (APP). (springer.com)
  • For example, a diffusible gradient is not suitable for the membrane/matrix proteins due to the difficulty of maintaining it over time. (cdc.gov)
  • Filamentous neurofibrillary tangles (NTF) are formed from paired helical filaments composed of hyperphosphorylated tau protein, a microtubule-associated protein. (springer.com)
  • To determine whether the deposition of the β-amyloid peptide (Aβ), a key pathological feature of Alzheimer's disease (AD), can be induced in vivo , we infused dilute supernatants of autopsy-derived neocortical homogenates from Alzheimer's patients unilaterally into the hippocampus and neocortex of 3-month-old β-amyloid precursor protein (βAPP)-transgenic mice. (jneurosci.org)
  • The results show that cerebral β-amyloid can be seeded in vivo by a single inoculation of dilute AD brain extract, demonstrating a key pathogenic commonality between β-amyloidosis and other neurodegenerative diseases involving abnormal protein polymerization. (jneurosci.org)
  • The ordered aggregation of fibrillogenic proteins into amyloid is most efficient above a critical protein concentration ( Harper and Lansbury, 1997 ). (jneurosci.org)
  • Plaques are formed mostly from the deposition of amyloid beta (Ab) a peptide derived from amyloid precursor protein (APP). (springer.com)
  • Lee VM, Otvos L Jr, Carden MJ, Hollosi M, Dietzschold B, Lazzarini RA: Identification of the major multiphosphorylation site in mammalian neurofilaments. (t3db.ca)
  • The colchicine-binding protein of mammalian brain and its relation to microtubules. (wikidata.org)
  • Actomyosin-like protein isolated from mammalian brain. (wikidata.org)
  • Here, we describe a pulse-spread fluorescence photoactivation method to address this in peripheral nerves dissected from hThy1-paGFP-NFM transgenic mice, which express a photoactivatable fluorescent neurofilament protein. (eneuro.org)
  • Neurofilament light (NF-L) levels are elevated in Alzheimer's Disease. (biolegend.com)
  • Nf are highly phosphorylated proteins, and the degree of this phosphorylation determines the axon diameter [ 6 ]. (biomedcentral.com)
  • Furthermore, the protein level of Bax was decreased whereas the protein levels of Bcl-2 and NF200 were increased after the GTP treatment. (hindawi.com)
  • Many neurodegenerative diseases are associated with the abnormal sequestration of disease-specific proteins in the brain, but the events that initiate this process remain unclear. (jneurosci.org)
  • IHC staining of HRP anti-Neurofilament L (NF-L) antibody (clone NFL3) on formalin-fixed paraffin-embedded human brain tissue. (biolegend.com)
  • However, these small strokes may result in the release of proteins that are associated with brain cell death, and these proteins can be measured in blood," he says. (dlife.com)
  • The discovery of specific genes and proteins associated with AD, and the development of new technologies for the production of transgenic animals, has helped researchers to overcome the lack of natural models. (springer.com)
  • C1q and C3 mRNA and protein levels were found elevated in spinal cord and engine cortex of individuals with sporadic ALS [15]. (healthcarecoremeasures.com)
  • The proximal and distal spread of the fluorescence due to the movement of the fluorescent neurofilaments was measured over time. (eneuro.org)
  • The method provides for an enhanced yield of purified protein and has markedly reduced admixture of electrophoresed protein with acrylamide and associated protein contaminants. (elsevier.com)
  • Peptide mapping of proteins from acrylamide gels. (wikidata.org)
  • The HSPB1 gene provides instructions for making a protein called heat shock protein beta-1 (also called heat shock protein 27). (medlineplus.gov)
  • The significance of the protein deposits per se for the pathogenesis of the diseases is debatable, but the universal tendency of the offending proteins to self-aggregate suggests that ordered protein polymerization is important in the pathogenesis of these disorders. (jneurosci.org)
  • The finding of large increases in the autophosphorylation and calmodulin-binding to CaM kinase II with only slight increases in the amount of antibody-binding suggests that CS2 exposure results in increased Ca2+/calmodulin-dependent phosphorylation of proteins by inducing an increase in kinase activity. (cdc.gov)
  • Mutations in the small heat-shock protein HSPB1 (HSP27) are responsible for one form of dHMN. (ox.ac.uk)
  • These findings support the neurofilament protein as a biomarker for disease activity in neurosarcoidosis, a form of sarcoidosis that affects the nervous system. (wijhebbensarcoidose.nl)
  • Abnormal CSF was found in 19 (95%) of those with neurosarcoidosis, including 16 with elevated immune cells and 17 with high protein levels. (wijhebbensarcoidose.nl)
  • Korley is the author of a new study that measured levels of a blood protein in patients with diabetes, who had not previously had a stroke, to predict their risk of experiencing a stroke in the future. (dlife.com)
  • High levels of the protein are present even before therapy begins, and those levels remain elevated throughout treatment and up to a month afterward. (clpmag.com)
  • Elevated or rising protein levels on serial lumbar punctures and 10 or fewer mononuclear cells/mm 3 strongly support the diagnosis. (medscape.com)
  • However, it is unclear whether neurofilament transport is also bidirectional in vivo . (eneuro.org)
  • Although it is thought to play a role in muscle contraction, the specific function of heat shock protein beta-1 in muscle cells is unclear. (medlineplus.gov)
  • Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20? (joplink.net)
  • One component of neurofilaments is the NFL protein, exclusively found in nerve cells. (wijhebbensarcoidose.nl)
  • This protein is a member of the heat shock protein family, which helps protect cells under adverse conditions such as infection, inflammation, exposure to toxins, elevated temperature, injury, and disease. (medlineplus.gov)
  • Kampinga HH, Garrido C. HSPBs: small proteins with big implications in human disease. (medlineplus.gov)
  • The relationship between lifestyle and serum neurofilament light protein in Huntington's disease. (cdc.gov)
  • Some terms (e.g.: blood) may appear in names of tissue, protein or disease or be included in an article title. (expasy.org)