Correlation Between Anti-Myelin Proteolipid Protein (PLP) Antibodies and Disease Severity in Multiple Sclerosis Patients With PLP Response-Permissive HLA Types. (nih.gov)
Additionally, we compare differences in chemokine expression patterns between wild-type Lewis rats that have an intact CNS, and transgenic Lewis rats that overexpress myelin proteolipid protein and that are characterized by myelin degeneration and myelin repair processes. (univie.ac.at)
Moreover, myelin-associated proteins, such as myelin basic protein (MBP), myelin proteolipid protein (PLP), and myelin oligodendrocyte glycoprotein (MOG), serve as antigens to induce an adaptive autoimmune response in MS patients [ 22 , 23 ]. (encyclopedia.pub)
Progenitor3
oligodendrocyte progenitor cells are not. (cornell.edu)
May regulate a critical transition point in oligodendrocyte lineage development by modulating oligodendrocyte progenitor proliferation relative to terminal differentiation and up-regulation of myelin gene transcription. (icr.ac.uk)
In vitro, the highest levels of PAR1 were observed in oligodendrocyte progenitor cells (OPCs), being reduced with differentiation. (elsevierpure.com)
Lineage6
2010) NG2+ CNS glial progenitors remain committed to the oligodendrocyte lineage in postnatal life and following neurodegeneration. (als.net)
iii) The effects of ε-toxin on the oligodendrocyte lineage are restricted to mature oligodendrocytes. (cornell.edu)
To gain insights into the impact of hypomyelination on excitatory and inhibitory neuronal network function in A1, we took advantage of mice in which ErbB receptor signaling in oligodendrocytes has been eliminated by expression of a dominant-negative ErbB4 in cells of the oligodendrocyte lineage under the control of the CNPase promoter (CNP-DN-ErbB4, 15 ). (biorxiv.org)
Double-labeling experiments with different markers of oligodendrocyte lineage such as olig1, olig2, platelet-derived growth factor receptor alpha, and proteolipid protein showed that Sema4D was expressed selectively by oligodendrocytes and myelin. (ox.ac.uk)
Albeit promising preclinical results demonstrated disease amelioration and remyelination enhancement via modulating oligodendrocyte lineage cells, most drug candidates showed only modest or no effects in human clinical trials. (encyclopedia.pub)
This might be due to the fact that remyelination is a sophistically orchestrated process that calls for the interplay between oligodendrocyte lineage cells, neurons, central nervous system (CNS) resident innate immune cells, and peripheral immune infiltrates and that this process may somewhat differ in humans and rodent models used in research. (encyclopedia.pub)
PLP15
With the use of transgenic and transfection paradigms, we have been able to show that the first intron of the PLP1 contains an enhancer region that is required for expression in oligodendrocytes, as well as in other cell types that express PLP1. (uams.edu)
Like X-linked Pelizaeus-Merzbacher disease (PMD, MIM: 312080), which is caused by mutations in the gene encoding proteolipid protein 1 ( PLP1 , MIM: 300401), one of the major proteins in the central nervous system (CNS) myelin, PMLD is characterized by impaired motor development resulting in nystagmus, dysarthria, progressive spasticity and ataxia. (prolekarniky.cz)
[ 1 ] is a congenital hypomyelination disorder caused by changes affecting the proteolipid protein 1 gene (PLP1) located on Xq22.2. (medscape.com)
Although Pelizaeus-Merzbacher disease and X-linked spastic paraplegia type 2 are nosologically distinguished, they are at opposite ends of a clinical spectrum of X-linked diseases caused by mutations of the same gene, the proteolipid protein 1 ( PLP1 ) gene, and result in defective central nervous system (CNS) myelination (see the image below). (medscape.com)
this provides strong experimental support for the hypothesis that overexpression of PLP1 is deleterious to oligodendrocytes. (medscape.com)
OPCs3
During development, oligodendrocyte precursor cells (OPCs) differentiate into oligodendrocytes (OLs) that extend processes to contact multiple axons of CNS neurons. (jneurosci.org)
Oligodendrocytes, the myelinating cells of the central nervous system (CNS), are generated from oligodendrocyte precursor cells (OPCs) that express neurotransmitter receptors. (nature.com)
Fig. 7: Spontaneous Ca 2+ transients in OPCs progressively decline as they differentiate into myelinating oligodendrocytes. (nature.com)
Neurons2
In primary CNS cultures, ε-toxin binds to and kills oligodendrocytes but not astrocytes, microglia, or neurons. (cornell.edu)
Oligodendrocytes are myelinating cells of the central nervous system which support functionally, structurally, and metabolically neurons. (biomedcentral.com)
Mature oligodendrocytes4
Clostridium perfringens Epsilon Toxin Causes Selective Death of Mature Oligodendrocytes and Central Nervous System Demyelination. (cornell.edu)
Mature oligodendrocytes are generally believed to be mere targets of destruction in the context of neuroinflammation and tissue damage, but their real degree of in vivo plasticity has become a matter of debate. (biomedcentral.com)
Cytoskeletal gene expression was quantified from mature oligodendrocytes directly isolated from transgenic animals through cell sorting. (biomedcentral.com)
Mature oligodendrocytes acutely increase their cytoskeletal plasticity in vivo during demyelination. (biomedcentral.com)
Axonal6
The encoded protein may play a role in the compaction, stabilization, and maintenance of myelin sheaths, as well as in oligodendrocyte development and axonal survival. (nih.gov)
Here, through the use of conditional knock-out mice, we demonstrate a requirement for ILK in oligodendrocyte differentiation and axonal myelination in vivo . (jneurosci.org)
In conjunction, ILK-deficient primary oligodendrocytes are defined by a failure in process extension and an inability to form myelin membrane upon axonal contact. (jneurosci.org)
2012) Glycolytic oligodendrocytes maintain myelin and long-term axonal integrity. (als.net)
1998) Axonal swellings and degeneration in mice lacking the major proteolipid of myelin. (als.net)
The transmembrane semaphorin Sema4D/CD100, an inhibitor of axonal growth, is expressed on oligodendrocytes and upregulated after CNS lesion. (ox.ac.uk)
Glial5
The neuropathological hallmark of MSA is the abnormal accumulation of α-synuclein in oligodendrocytes forming glial cytoplasmic inclusions. (frontiersin.org)
MSA is characterized by an abnormal accumulation of α-synuclein forming glial cytoplasmic inclusions mainly in oligodendrocytes ( 2 , 3 ) and thus designated as α-synucleinopathy ( 4 ). (frontiersin.org)
ε-Toxin specificity for oligodendrocytes was confirmed using enriched glial culture. (cornell.edu)
10-15-day-old homozygous Cx47M282T and Cx47 null mice revealed a more than 80% reduction in the number of cells participating in glial networks after biocytin injections into oligodendrocytes in sections of corpus callosum. (prolekarniky.cz)
In contrast to the tumor parent, M03.13 expressed surface immunoreactivity for galactosyl cerebroside(GS) and intracellular immunoreactivity for myelin basic protein (MBP), proteolipid protein (PLP), and glial fibrillary acidic protein (GFAP). (tebubio.com)
Cells10
Oligodendrocytes supply nerve cells with energy-rich metabolites such as lactate to help keep the power on. (als.net)
We used compound heterozygote mice to study the long-term fate of oligodendrocytes expressing either the jimpy or rumpshaker allele against a background of cells expressing a Plp -null allele. (rupress.org)
Although mutant and null oligodendrocytes were generated in equal numbers, the proportion expressing the mutant allele subsequently declined, but whereas those expressing the rumpshaker allele formed a reduced but stable population, the number of jimpy cells fell progressively. (rupress.org)
C57BL/6 (B6) mice deficient for the class II transactivator, which have defects in MHC class II, invariant chain (Ii), and H-2M (DM) expression, are resistant to initiation of myelin oligodendrocyte protein (MOG) peptide, MOG 35-55 -specific EAE by both priming and adoptive transfer of encephalitogenic T cells. (aai.org)
Oligodendrocytes are a type of specialized cells found in the central nervous system (CNS) that play a crucial role in the formation and maintenance of myelin, further supporting its potential role in promoting myelin repair. (veterans4christ.org)
We find that ε-toxin specifically targets the myelin-forming cells of the central nervous system (CNS), oligodendrocytes, leading to cell death. (cornell.edu)
The selectivity of ε-toxin for oligodendrocytes is remarkable, as other cells of the CNS are unaffected. (cornell.edu)
Sema4D-positive cells were not colabeled with the astrocyte marker GFAP, with the microglial and macrophagic marker isolectin B4, or with NG2, a marker of oligodendrocyte precursors. (ox.ac.uk)
Oligodendrocytes, the myelinating cells of central nervous system, have extraordinary demands for oxygen, nutrients, and metabolic substrates associated with production of myelin protein and proteolipid as well as axonotrophic support. (chavalilab.com)
The inflammatory substances released by autoreactive T-cells, specific for myelin antigens, such as Myelin Oligodendrocyte Glycoprotein (MOG), damage and scar the sheaths, as well as the oligodendrocytes (myelinating cells of the CNS) and underlying nerves, eventually. (sb-peptide.com)
Microglia3
For this classification of MS lesions, identification of myelin with histological stains [such as luxol fast blue-PAS] or by immunohistochemistry using antibodies against myelin basic-protein (MBP) or proteolipid-protein (PLP), as well as, detection of macrophages/microglia by, e.g., anti-CD68 is sufficient. (nih.gov)
Adult mCx47M282T expressing mice did not show substantial myelin alterations, but homozygous Cx47M282T mice, additionally deprived of connexin32, which is also expressed in oligodendrocytes, died within six weeks after birth and displayed severe myelin defects accompanied by astrogliosis and activated microglia. (prolekarniky.cz)
These cytokines can directly attack myelinating oligodendrocytes (OLs) [ 2 ] or indirectly impair the OL-neuron coupling by polarizing microglia into the M1 (pro-inflammatory) state, which then activate reactive neurotoxic A1 astrocytes by secreting Il-1α, TNFα, and the complement component 1q (C1q) [ 3 ]. (encyclopedia.pub)
Differentiation1
Additionally, differentiation, forskolin holds great potential as a compound for promoting myelin repair in oligodendrocytes. (veterans4christ.org)
Gene6
This gene encodes a transmembrane proteolipid protein that is the predominant component of myelin. (nih.gov)
Mutations in the X-linked Plp gene lead to dysmyelinating phenotypes and oligodendrocyte cell death. (rupress.org)
Expression of the gene is regulated spatiotemporally in oligodendrocytes, with peak expression occurring during the active myelination period of CNS development. (uams.edu)
Human oligodendrocytes express the gap junction protein connexin47 (Cx47), which is encoded by the GJC2 gene. (prolekarniky.cz)
Activity of this promoter served as surrogate for dynamics of the cytoskeleton gene transcription through recording of in vivo bioluminescence following diphtheria toxin-induced oligodendrocyte death and autoimmune demyelination. (biomedcentral.com)
Specifically, PAR1 gene deletion resulted in earlier onset of spinal cord myelination, including substantially more Olig2-positive oligodendrocytes, more myelinated axons, and higher proteolipid protein (PLP) levels at birth. (elsevierpure.com)
Promoter3
Mice overexpressing human α-synuclein under the control of the oligodendrocyte specific proteolipid promoter (PLP-α-syn) display aged-dependent progressive neurodegeneration and have been extensively used in the past to assess MSA-related alterations ( 16 - 19 ). (frontiersin.org)
We used a novel mouse model (oLucR) expressing luciferase in myelin oligodendrocyte glycoprotein-positive oligodendrocytes under the control of a β-actin promoter. (biomedcentral.com)
oligodendrocytes are severely malformed in vitro (a relatively simple environment), but undergo phenotypic recovery in the context of the whole animal. (jneurosci.org)
In vitro, only a small number of primary oligodendrocytes express GFAP and GFAP+ astocytes rarely express oligodendrocytic markers. (tebubio.com)
MO3.13, an immortal human-human hybrid cell line that express phenotypic characteristics of primary oligodendrocytes, was created by fusing a 6-thioguanine-resistant mutant of the human rhabdomyosarcoma RD with adult human oligodendrocytes by a lectin-enhanced polyethylene glycol procedure. (tebubio.com)
Cyclic nucleotide1
The former is defined by the presence in macrophages of major and small molecular weight myelin proteins, such as cyclic nucleotide diphosphoesterase (CNP), myelin oligodendrocyte glycoprotein (MOG), or myelin-associated protein (MAG), whereas macrophages in the latter demonstrate merely the presence of the major myelin proteins MBP or PLP. (nih.gov)
Demyelination3
A neutralizing antibody against ε-toxin inhibits oligodendrocyte death and demyelination. (cornell.edu)
Importantly, ε-toxin-induced oligodendrocyte death results in demyelination and is dependent on expression of myelin and lymphocyte protein (MAL). (cornell.edu)
To model the white matter pathology seen in MS, we use both acute and chronic demyelination injuries as well as a genetically induced oligodendrocyte cell death model. (chavalilab.com)
Mice2
This phenotype is not Ag-specific, as DM- and Ii-deficient mice are also resistant to initiation of EAE by proteolipid protein peptide PLP 178-191 . (aai.org)
To address this gap in knowledge, we studied mice that have mild hypomyelination due to loss of oligodendrocyte ErbB receptor signaling. (biorxiv.org)
Primary1
Primary oligodendrocytes from rat brain that have been differentiated for 2.5 days. (pabmabs.com)
Selective1
i) ε-Toxin causes selective oligodendrocyte death while preserving all other neural elements. (cornell.edu)
Cellular1
Together, our work demonstrates ILK as necessary for normal oligodendrocyte development, reinforces its role as a bridge between the actin cytoskeleton and cell membrane, and highlights the overarching compensatory capacity of oligodendrocytes in response to cellular milieu. (jneurosci.org)
Spinal1
At 8 d after spinal cord lesions, Sema4D expression was strongly upregulated in oligodendrocytes at the periphery of the lesion. (ox.ac.uk)
Expression4
Additionally, obesity, forskolin treatment was shown to increase the expression of MBP and PLP in cultured oligodendrocytes, and cardiovascular disorders. (veterans4christ.org)
ε-Toxin sensitivity is also dependent on oligodendrocyte expression of the proteolipid myelin and lymphocyte protein (MAL), as MAL-deficient oligodendrocytes are insensitive to ε-toxin. (cornell.edu)
iv) Expression of the developmentally regulated proteolipid MAL is required for the cytotoxic effects. (cornell.edu)
Sema4D expression in myelinating oligodendrocytes was further observed using neuron-oligodendrocyte cocultures. (ox.ac.uk)
Compound1
In compound heterozygotes expressing rumpshaker and jimpy alleles, oligodendrocytes expressing the former predominated and were more abundant than when the rumpshaker and null alleles were in competition. (rupress.org)
Transcription1
Increase in bioluminescence upon autoimmune inflammation was parallel to an oligodendrocyte-specific increased transcription of β-tubulin. (biomedcentral.com)
Survival1
Here, we exploit the X inactivation phenomenon to show that a hierarchy exists in the influence of different mutant Plp alleles on oligodendrocyte survival. (rupress.org)
Vivo1
Experimental demyelinating setups augmented oligodendrocyte-specific in vivo bioluminescence. (biomedcentral.com)
Human1
SB-PEPTIDE offers Myelin Oligodendrocyte Glycoprotein pool composed of 59 15-mer sequences with 11 amino acids overlap, covering the entire sequence of the human protein. (sb-peptide.com)
Experimental1
ALS Today 's Michelle Pflumm PhD talked to Max Planck Institute of Experimental Medicine neuroscientist Klaus-Armin Nave PhD about oligodendrocytes, their emerging role in ALS and potential treatment strategies going forward. (als.net)