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Myelin protein

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https://www.readbyqxmd.com/read/28634469/alexander-disease-mutations-produce-cells-with-coexpression-of-glial-fibrillary-acidic-protein-and-ng2-in-neurosphere-cultures-and-inhibit-differentiation-into-mature-oligodendrocytes
#1
Ulises Gómez-Pinedo, Maria Salomé Sirerol-Piquer, María Durán-Moreno, José Manuel García-Verdugo, Jorge Matias-Guiu
BACKGROUND: Alexander disease (AxD) is a rare disease caused by mutations in the gene encoding glial fibrillary acidic protein (GFAP). The disease is characterized by presence of GFAP aggregates in the cytoplasm of astrocytes and loss of myelin. OBJECTIVES: Determine the effect of AxD-related mutations on adult neurogenesis. METHODS: We transfected different types of mutant GFAP into neurospheres using the nucleofection technique. RESULTS: We find that mutations may cause coexpression of GFAP and NG2 in neurosphere cultures, which would inhibit the differentiation of precursors into oligodendrocytes and thus explain the myelin loss occurring in the disease...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28632137/electrospun-silk-polyaniline-conduits-for-functional-nerve-regeneration-in-rat-sciatic-nerve-injury-model
#2
Suradip Das, Manav Sharma, Dhiren Saharia, Kushal Sarma, Elizabeth Muir, Utpal Bora
The present study describes the fabrication of polyaniline-silk fibroin nanocomposite based nerve conduits and its subsequent implantation in a rat sciatic nerve injury model for peripheral nerve regeneration. This is the first in vivo study of polyaniline based nerve conduit describing the safety and efficacy of the conduits in treating peripheral nerve injuries. The nanocomposite was synthesised by electrospinning a mixture of silk fibroin protein and polyaniline wherein the silk nanofibers were observed to be uniformly coated with polyaniline nanoparticles...
June 20, 2017: Biomedical Materials
https://www.readbyqxmd.com/read/28631553/administration-of-progesterone-throughout-pregnancy-increases-maternal-steroids-without-adverse-effect-on-mature-oligodendrocyte-immunostaining-in-the-guinea-pig
#3
Julia C Shaw, Hannah K Palliser, Kerrin Palazzi, Jonathan J Hirst
Progesterone is administered to pregnant women at risk of premature labor, despite systematic reviews showing conflicting outcomes regarding its use, highlighting doubt over the effectiveness of the therapy. Progesterone can be rapidly metabolized into a number of steroids, but to date, there has been a lack of investigation into the fetal steroid profiles following administration and whether this impacts fetal neurodevelopment. The objective of this study was to determine the effect of progesterone treatment on allopregnanolone and cortisol levels in the fetus and on a marker of myelination in the fetal brain...
January 1, 2017: Reproductive Sciences
https://www.readbyqxmd.com/read/28631093/myelin-regulatory-factor-drives-remyelination-in-multiple-sclerosis
#4
Greg J Duncan, Jason R Plemel, Peggy Assinck, Sohrab B Manesh, Fraser G W Muir, Ryan Hirata, Matan Berson, Jie Liu, Michael Wegner, Ben Emery, G R Wayne Moore, Wolfram Tetzlaff
Remyelination is limited in the majority of multiple sclerosis (MS) lesions despite the presence of oligodendrocyte precursor cells (OPCs) in most lesions. This observation has led to the view that a failure of OPCs to fully differentiate underlies remyelination failure. OPC differentiation requires intricate transcriptional regulation, which may be disrupted in chronic MS lesions. The expression of few transcription factors has been differentially compared between remyelinating lesions and lesions refractory to remyelination...
June 19, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28629798/proteomic-analysis-of-mesenchymal-to-schwann-cell-transdifferentiation
#5
Anup D Sharma, Jayme Wiederin, Metin Uz, Pawel Ciborowski, Surya K Mallapragada, Howard E Gendelman, Donald S Sakaguchi
While transplantation of Schwann cells facilitates axon regeneration, remyelination and repair after peripheral nerve injury clinical use is limited by cell bioavailability. We posit that such limitation in cell access can be overcome by the use of autologous bone-marrow derived mesenchymal stem cells (MSCs). As MSCs can transdifferentiate to Schwann cell-phenotypes and accelerate nerve regeneration we undertook proteomic evaluation of the cells to uncover the protein contents that affects Schwann cell formulation...
June 16, 2017: Journal of Proteomics
https://www.readbyqxmd.com/read/28623606/marcks-is-necessary-for-oligodendrocyte-precursor-cell-maturation
#6
Zhao-Huan Zhang, Fan-Fei Ma, Hui Zhang, Xiao-Hui Xu
Oligodendrocyte precursor cell (OPC) development into myelinated oligodendrocytes demands vigorous membrane addition. Since myristoylated alanine-rich C-kinase substrate (MARCKS) reportedly contributes to Ras-associated protein (Rab)-10-associated vesicle insertion into neuronal membranes, we investigated the role of MARCKS in OPC maturation. We found that either knockdown of MARCKS or interruption of its interaction with Rab10 would cause a decrease of the cell membrane area during OPC development. Enhanced MARCKS phosphorylation by Nogo66 or myelin debris treatment inhibited OPC maturation, while its dephosphorylation by protein phosphatase 2 A activator D-erythro-sphingosine promoted OPC development in the presence of myelin debris...
June 16, 2017: Neurochemical Research
https://www.readbyqxmd.com/read/28620864/adipose-tissue-derived-stromal-cells-adsc-express-oligodendrocyte-and-myelin-markers-but-they-do-not-function-as-oligodendrocytes
#7
Lara Vellosillo, Maria Paz Muñoz, Carlos Luis Paíno
Mesenchymal cells cultured from the vasculo-stromal fraction of adipose tissue (ADSC) show adult stem cell characteristics and several groups have claimed generating neural cells from them. However, we have observed that many markers commonly used for the identification of neural cells are spontaneously expressed by ADSC in culture. In the present study, we have examined the expression of characteristic oligodendrocyte molecules in cultured ADSC, aiming to test if myelinating cells could be generated from accessible non-neural adult tissues...
June 15, 2017: Histochemistry and Cell Biology
https://www.readbyqxmd.com/read/28618115/iron-sulfur-glutaredoxin-2-protects-oligodendrocytes-against-damage-induced-by-nitric-oxide-release-from-activated-microglia
#8
Klaudia Lepka, Katrin Volbracht, Eckhard Bill, Reiner Schneider, Natalia Rios, Thomas Hildebrandt, Jens Ingwersen, Timur Prozorovski, Christopher Horst Lillig, Jack van Horssen, Lawrence Steinman, Hans-Peter Hartung, Rafael Radi, Arne Holmgren, Orhan Aktas, Carsten Berndt
Demyelinated brain lesions, a hallmark of autoimmune neuroinflammatory diseases like multiple sclerosis, result from oligodendroglial cell damage. Activated microglia are considered a major source of nitric oxide and subsequent peroxynitrite-mediated damage of myelin. Here, we provide biochemical and biophysical evidence that the oxidoreductase glutaredoxin 2 inhibits peroxynitrite formation by transforming nitric oxide into dinitrosyl-diglutathionyl-iron-complexes. Glutaredoxin 2 levels influence both survival rates of primary oligodendrocyte progenitor cells and preservation of myelin structure in cerebellar organotypic slice cultures challenged with activated microglia or nitric oxide donors...
June 15, 2017: Glia
https://www.readbyqxmd.com/read/28617998/schwann-cell-specific-deletion-of-the-endosomal-pi-3-kinase-vps34-leads-to-delayed-radial-sorting-of-axons-arrested-myelination-and-abnormal-erbb2-erbb3-tyrosine-kinase-signaling
#9
Anne M Logan, Anna E Mammel, Danielle C Robinson, Andrea L Chin, Alec F Condon, Fred L Robinson
The PI 3-kinase Vps34 (Pik3c3) synthesizes phosphatidylinositol 3-phosphate (PI3P), a lipid critical for both endosomal membrane traffic and macroautophagy. Human genetics have implicated PI3P dysregulation, and endosomal trafficking in general, as a recurring cause of demyelinating Charcot-Marie-Tooth (CMT) peripheral neuropathy. Here, we investigated the role of Vps34, and PI3P, in mouse Schwann cells by selectively deleting Vps34 in this cell type. Vps34-Schwann cell knockout (Vps34(SCKO) ) mice show severe hypomyelination in peripheral nerves...
June 15, 2017: Glia
https://www.readbyqxmd.com/read/28616777/differential-regulation-of-mecp2-phosphorylation-by-laminin-in-oligodendrocytes
#10
Zalak S Parikh, Ashutosh Tripathi, Prakash P Pillai
Oligodendrocytes (OLGs) are the myelinating cells of the central nervous system (CNS), and its proper differentiation is crucial for normal functioning of neurons. Methyl-CpG-binding protein 2 (MeCP2) is a multifunctional methylated DNA binding protein; mutation of which causes Rett syndrome, a severe neurodevelopmental disorder. Previously, we reported that MeCP2 is expressed in all the stages of oligodendrocyte development, and also shown the role of MeCP2 as a transcription regulator of myelin genes in OLGs...
June 14, 2017: Journal of Molecular Neuroscience: MN
https://www.readbyqxmd.com/read/28616446/treatment-with-anti-fc%C3%AE%C2%B5ri%C3%AE-antibody-exacerbates-eae-and-t-cell-immunity-against-myelin
#11
Silvia Musio, Massimo Costanza, Pietro Luigi Poliani, Elena Fontana, Manuela Cominelli, Gabriella Abolafio, Lawrence Steinman, Rosetta Pedotti
OBJECTIVE: To investigate the effects of targeting the high-affinity receptor for immunoglobulin E (FcεRI), that plays a central role in allergic responses and is constitutively expressed on mast cells and basophils, in clinical disease and autoimmune T-cell response in experimental MS. METHODS: Experimental autoimmune encephalomyelitis (EAE) was induced in C57BL/6 mice by immunization with myelin oligodendrocyte glycoprotein 35-55. Anti-FcεRI α-chain antibody was administered intraperitoneally...
May 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28612133/brain-specific-serum-biomarkers-predict-neurological-morbidity-in-diagnostically-diverse-pediatric-intensive-care-unit-patients
#12
Alicia K Au, Michael J Bell, Ericka L Fink, Rajesh K Aneja, Patrick M Kochanek, Robert S B Clark
BACKGROUND: Unexpected neurological morbidity in Pediatric Intensive Care Units (PICUs) remains high and is difficult to detect proactively. Brain-specific biomarkers represent a novel approach for early detection of neurological injury. We sought to determine whether serum concentrations of neuron-specific enolase (NSE), myelin basic protein (MBP), and S100B, specific for neurons, oligodendrocytes, and glia, respectively, were predictive of neurological morbidity in critically ill children...
June 13, 2017: Neurocritical Care
https://www.readbyqxmd.com/read/28604632/experimental-autoimmune-encephalomyelitis-eae-induced-elevated-expression-of-the-e1-isoform-of-methyl-cpg-binding-protein-2-mecp2e1-implications-in-multiple-sclerosis-ms-induced-neurological-disability-and-associated-myelin-damage
#13
Tina Khorshid Ahmad, Ting Zhou, Khaled AlTaweel, Claudia Cortes, Ryan Lillico, Ted Martin Lakowski, Kiana Gozda, Michael Peter Namaka
Multiple sclerosis (MS) is a chronic neurological disease characterized by the destruction of central nervous system (CNS) myelin. At present, there is no cure for MS due to the inability to repair damaged myelin. Although the neurotrophin brain derived neurotrophic factor (BDNF) has a beneficial role in myelin repair, these effects may be hampered by the over-expression of a transcriptional repressor isoform of methyl CpG binding protein 2 (MeCP2) called MeCP2E1. We hypothesize that following experimental autoimmune encephalomyelitis (EAE)-induced myelin damage, the immune system induction of the pathogenic MeCP2E1 isoform hampers the myelin repair process by repressing BDNF expression...
June 12, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28598205/neurofilament-light-chain-protein-as-a-marker-of-neuronal-injury-review-of-its-use-in-hiv-1-infection-and-reference-values-for-hiv-negative-controls
#14
Aylin Yilmaz, Kaj Blennow, Lars Hagberg, Staffan Nilsson, Richard W Price, Judith Schouten, Serena Spudich, Jonathan Underwood, Henrik Zetterberg, Magnus Gisslén
Several CSF biomarkers of neuronal injury have been studied in people living with HIV. At this time, the most useful is the light subunit of the neurofilament protein (NFL). This major structural component of myelinated axons is essential to maintain axonal caliber and to facilitate effective nerve conduction. CSF concentrations of NFL provide a sensitive marker of CNS injury in a number of neurological diseases, including HIV-related neuronal injury. Areas Covered: In this review, the authors describe CSF NFL concentrations across the spectrum of HIV-infection, from its early acute phase to severe immunosuppression, with and without neurological conditions, and with and without antiretroviral treatment (n = 516)...
June 14, 2017: Expert Review of Molecular Diagnostics
https://www.readbyqxmd.com/read/28597407/resistance-wheel-exercise-from-mid-life-has-minimal-effect-on-sciatic-nerves-from-old-mice-in-which-sarcopenia-was-prevented
#15
Vidya S Krishnan, Zoe White, Jessica R Terrill, Stuart I Hodgetts, Melinda Fitzgerald, Tea Shavlakadze, Alan R Harvey, Miranda D Grounds
The ability of resistance exercise, initiated from mid-life, to prevent age-related changes in old sciatic nerves, was investigated in male and female C57BL/6J mice. Aging is associated with cellular changes in old sciatic nerves and also loss of skeletal muscle mass and function (sarcopenia). Mature adult mice aged 15 months (M) were subjected to increasing voluntary resistance wheel exercise (RWE) over a period of 8 M until 23 M of age. This prevented sarcopenia in the old 23 M aged male and female mice...
June 8, 2017: Biogerontology
https://www.readbyqxmd.com/read/28594400/the-role-of-oligodendrocyte-precursor-cells-expressing-the-gpr17-receptor-in-brain-remodeling-after-stroke
#16
Elisabetta Bonfanti, Paolo Gelosa, Marta Fumagalli, Leda Dimou, Francesca Viganò, Elena Tremoli, Mauro Cimino, Luigi Sironi, Maria P Abbracchio
Following stroke-induced neuronal damage, quiescent oligodendrocyte precursors (OPCs) are activated to proliferate and later to differentiate to myelin-producing cells. GPR17, a receptor transiently expressed on early OPCs, has emerged as a target to implement stroke repair through stimulation of OPC maturation. However, being GPR17 completely downregulated in myelin-producing oligodendrocytes, its actual role in determining the final fate of OPCs after cerebral ischemia is still uncertain. Here, to univocally define the spatiotemporal changes and final fate of GPR17-expressing OPCs, we induced ischemia by middle cerebral artery occlusion (MCAo) in reporter GPR17iCreER(T2):CAG-eGreen florescent protein (GFP) mice, in which, upon tamoxifen treatment, cells expressing GPR17 become green and traceable for their entire life...
June 8, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28594344/design-and-synthesis-of-non-peptide-mimetics-mapping-the-immunodominant-myelin-basic-protein-mbp83-96-epitope-to-function-as-t-cell-receptor-antagonists
#17
Mary-Patricia Yannakakis, Carmen Simal, Haralambos Tzoupis, Maria Rodi, Narges Dargahi, Monica Prakash, Athanasia Mouzaki, James A Platts, Vasso Apostolopoulos, Theodore V Tselios
Encephalitogenic T cells are heavily implicated in the pathogenesis of multiple sclerosis (MS), an autoimmune demyelinating disease of the central nervous system. Their stimulation is triggered by the formation of a trimolecular complex between the human leukocyte antigen (HLA), an immunodominant myelin basic protein (MBP) epitope, and the T cell receptor (TCR). We detail herein our studies directed towards the rational design and synthesis of non-peptide mimetic molecules, based on the immunodominant MBP83-96 epitope that is recognized by the TCR in complex with HLA...
June 8, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28594066/novel-biallelic-mutations-in-the-pnpt1-gene-encoding-a-mitochondrial-rna-import-protein-pnpase-cause-delayed-myelination
#18
Ryo Sato, Natsuko Arai-Ichinoi, Atsuo Kikuchi, Tetsuro Matsuhashi, Yurika Numata-Uematsu, Mitsugu Uematsu, Yuji Fujii, Kei Murayama, Akira Ohtake, Takaaki Abe, Shigeo Kure
Recent studies suggest that impaired transcription or mitochondrial translation of small RNAs can cause abnormal myelination. A polynucleotide phosphorylase (PNPase) encoded by PNPT1 facilitates the import of small RNAs into mitochondria. PNPT1 mutations have been reported in patients with neurodevelopmental diseases with mitochondrial dysfunction. We report here two siblings with PNPT1 mutations who presented delayed myelination as well as mitochondrial dysfunction. We identified compound heterozygous mutations (c...
June 8, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28592428/ebv-infection-empowers-human-b-cells-for-autoimmunity-role-of-autophagy-and-relevance-to-multiple-sclerosis
#19
Elena Morandi, S Anwar Jagessar, Bert A 't Hart, Bruno Gran
The efficacy of B cell depletion therapy in multiple sclerosis indicates their central pathogenic role in disease pathogenesis. The B lymphotropic EBV is a major risk factor in multiple sclerosis, via as yet unclear mechanisms. We reported in a nonhuman primate experimental autoimmune encephalomyelitis model that an EBV-related lymphocryptovirus enables B cells to protect a proteolysis-sensitive immunodominant myelin oligodendrocyte glycoprotein (MOG) epitope (residues 40-48) against destructive processing...
June 7, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28586011/microglia-activation-triggers-oligodendrocyte-precursor-cells-apoptosis-via-hsp60
#20
Yunhong Li, Rui Zhang, Xiaolin Hou, Yumei Zhang, Feijia Ding, Fan Li, Yao Yao, Yin Wang
Reactive microglia are present in lesions of myelin‑associated white matter disorders resulting in injuries to oligodendrocyte precursor cells (OPCs). Therefore, protection of OPCs from injury due to excessive activation of microglia is important in treating these diseases. Heat shock protein 60 (HSP60) has been demonstrated to be released extracellularly in the failing heart upon stress or injury. However, the role of HSP60 in the central nervous system and whether it participates in the toxic effects of microglia on OPCs remains unclear...
July 2017: Molecular Medicine Reports
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