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https://www.readbyqxmd.com/read/29348134/cerebral-mitochondrial-microangiopathy-leads-to-leukoencephalopathy-in-mitochondrial-neurogastrointestinal-encephalopathy
#1
L L Gramegna, A Pisano, C Testa, D N Manners, R D'Angelo, E Boschetti, F Giancola, L Pironi, L Caporali, M Capristo, M L Valentino, G Plazzi, C Casali, M T Dotti, G Cenacchi, M Hirano, C Giordano, P Parchi, R Rinaldi, R De Giorgio, R Lodi, V Carelli, C Tonon
BACKGROUND AND PURPOSE: Mitochondrial neurogastrointestinal encephalopathy is a rare disorder due to recessive mutations in the thymidine phosphorylase gene, encoding thymidine phosphorylase protein required for mitochondrial DNA replication. Clinical manifestations include gastrointestinal dysmotility and diffuse asymptomatic leukoencephalopathy. This study aimed to elucidate the mechanisms underlying brain leukoencephalopathy in patients with mitochondrial neurogastrointestinal encephalopathy by correlating multimodal neuroradiologic features to postmortem pathology...
January 18, 2018: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/29347989/bal%C3%A3-s-concentric-sclerosis-is-immunologically-distinct-from-multiple-sclerosis-results-from-retrospective-analysis-of-almost-150-lumbar-punctures
#2
S Jarius, C Würthwein, J R Behrens, J Wanner, J Haas, F Paul, B Wildemann
BACKGROUND: Baló's concentric sclerosis (BCS) is a rare inflammatory demyelinating disorder of the central nervous system characterised by concentric layers of demyelination. It is unclear whether BCS is a variant of multiple sclerosis (MS) or a disease entity in its own right. OBJECTIVE: To compare the cerebrospinal fluid (CSF) features of BCS to those of MS. METHODS: Retrospective analysis of the CSF profile of all patients with BCS reported in the medical literature between 1980 and 2017...
January 18, 2018: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/29344669/clc-2-is-a-positive-modulator-of-oligodendrocyte-precursor-cell-differentiation-and-myelination
#3
Xiaolin Hou, Rui Zhang, Junyan Wang, Yunhong Li, Fan Li, Yan Zhang, Xiaomin Zheng, Ying Shen, Yin Wang, Liang Zhou
Oligodendrocytes (OLs) are myelin-forming cells that are present within the central nervous system. Impaired oligodendrocyte precursor cell (OPC) differentiation into mature OLs is a major cause of demyelination diseases. Therefore, identifying the underlying molecular mechanisms of OPC differentiation is crucial to understand the processes of myelination and demyelination. It has been acknowledged that various extrinsic and intrinsic factors are involved in the control of OPC differentiation; however, the function of ion channels, particularly the voltage‑gated chloride channel (CLC), in OPC differentiation and myelination are not fully understood...
January 17, 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29340845/dihydrotestosterone-treatment-accelerates-autograft-reversal-sciatic-nerve-regeneration-in-rats
#4
Xiaofan Yang, Pingping Xue, Ruozheng Wei, Xin Liu, Xiang Xu, Zhenyu Liu, Yanhua Chen, Zhenbing Chen
Neuroactive steroids such as progesterone, testosterone, and their derivatives have been widely studied for their neuroprotective roles in the nervous system. Autologous nerve transplantation is considered as the gold standard repair technique when primary suture is impossible; nevertheless, this method is far from ideal. In this study, we aimed to explore the impact of dihydrotestosterone (DHT), a 5α-reduced derivative of testosterone, on the recovery of peripheral nerve injury treated with autologous nerve transplantation...
January 16, 2018: Neurochemical Research
https://www.readbyqxmd.com/read/29339314/surface-based-characteristics-of-the-cerebellar-cortex-visualized-with-ultra-high-field-mri
#5
Yohan Boillat, Pierre-Louis Bazin, Kieran O'Brien, Mário João Fartaria, Guillaume Bonnier, Gunnar Krueger, Wietske Van der Zwaag, Cristina Granziera
Although having a relatively homogeneous cytoarchitectonic organization, the cerebellar cortex is a heterogeneous region characterized by different amounts of myelin, iron and protein expression profiles. In this study, we used quantitative T1 and T2* mapping at ultra-high field (7T) MRI to investigate the tissue characteristics of the cerebellar gray matter surface and its layers. Detailed subject-specific surfaces were generated at three different cortical depths and averaged across subjects to create averaged T1 and T2* maps on the cerebellar surface...
January 12, 2018: NeuroImage
https://www.readbyqxmd.com/read/29337237/uridine-5-triphosphate-partially-blocks-differentiation-signals-and-favors-a-more-repair-state-in-cultured-rat-schwann-cells
#6
Marta Palomo-Guerrero, Jose Miguel Cosgaya, Alejandro Gella, Núria Casals, Carmen Grijota-Martinez
Schwann cells (SCs) play a key role in peripheral nerve regeneration. After damage, they respond acquiring a repair phenotype that allows them to proliferate, migrate and redirect axonal growth. Previous studies have shown that Uridine-5'-Triphosphate (UTP) and its purinergic receptors participate in several pathophysiological responses in the nervous system. Our group has previously described how UTP induces the migration of a Schwannoma cell line and promotes wound healing. These data suggest that UTP participates in the signaling involved in the regeneration process...
January 11, 2018: Neuroscience
https://www.readbyqxmd.com/read/29337119/sox10-single-transcription-factor-based-fast-and-efficient-generation-of%C3%A2-oligodendrocytes-from-human-pluripotent-stem-cells
#7
Juan Antonio García-León, Manoj Kumar, Ruben Boon, David Chau, Jennifer One, Esther Wolfs, Kristel Eggermont, Pieter Berckmans, Nilhan Gunhanlar, Femke de Vrij, Bas Lendemeijer, Benjamin Pavie, Nikky Corthout, Steven A Kushner, José Carlos Dávila, Ivo Lambrichts, Wei-Shou Hu, Catherine M Verfaillie
Scarce access to primary samples and lack of efficient protocols to generate oligodendrocytes (OLs) from human pluripotent stem cells (hPSCs) are hampering our understanding of OL biology and the development of novel therapies. Here, we demonstrate that overexpression of the transcription factor SOX10 is sufficient to generate surface antigen O4-positive (O4+) and myelin basic protein-positive OLs from hPSCs in only 22 days, including from patients with multiple sclerosis or amyotrophic lateral sclerosis. The SOX10-induced O4+ population resembles primary human OLs at the transcriptome level and can myelinate neurons in vivo...
January 10, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/29337114/pathological-endogenous-%C3%AE-synuclein-accumulation-in-oligodendrocyte-precursor-cells-potentially-induces-inclusions-in-multiple-system-atrophy
#8
Seiji Kaji, Takakuni Maki, Hisanori Kinoshita, Norihito Uemura, Takashi Ayaki, Yasuhiro Kawamoto, Takahiro Furuta, Makoto Urushitani, Masato Hasegawa, Yusuke Kinoshita, Yuichi Ono, Xiaobo Mao, Tran H Quach, Kazuhiro Iwai, Valina L Dawson, Ted M Dawson, Ryosuke Takahashi
Glial cytoplasmic inclusions (GCIs), commonly observed as α-synuclein (α-syn)-positive aggregates within oligodendrocytes, are the pathological hallmark of multiple system atrophy. The origin of α-syn in GCIs is uncertain; there is little evidence of endogenous α-syn expression in oligodendrocyte lineage cells, oligodendrocyte precursor cells (OPCs), and mature oligodendrocytes (OLGs). Here, based on in vitro analysis using primary rat cell cultures, we elucidated that preformed fibrils (PFFs) generated from recombinant human α-syn trigger multimerization and an upsurge of endogenous α-syn in OPCs, which is attributable to insufficient autophagic proteolysis...
December 27, 2017: Stem Cell Reports
https://www.readbyqxmd.com/read/29327207/phenotypic-and-functional-characteristics-of-human-schwann-cells-as-revealed-by-cell-based-assays-and-rna-seq
#9
Paula V Monje, David Sant, Gaofeng Wang
This study comprehensively addresses the phenotype, function, and whole transcriptome of primary human and rodent Schwann cells (SCs) and highlights key species-specific features beyond the expected donor variability that account for the differential ability of human SCs to proliferate, differentiate, and interact with axons in vitro. Contrary to rat SCs, human SCs were insensitive to mitogenic factors other than neuregulin and presented phenotypic variants at various stages of differentiation, along with a mixture of proliferating and senescent cells, under optimal growth-promoting conditions...
January 11, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29325876/nogo-a-interacts-with-trka-to-alter-nerve-growth-factor-signaling-in-nogo-a-overexpressing-pc12-cells
#10
Robert G Farrer, Gwendolyn L Kartje
The Nogo-A protein, originally discovered as a potent myelin-associated inhibitor of neurite outgrowth, is also expressed by certain neurons, especially during development and after injury, but its role in neuronal function is not completely known. In this report, we overexpressed Nogo-A in PC12 cells to use as a model to identify potential neuronal signaling pathways affected by endogenously expressed Nogo-A. Unexpectedly, our results show that viability of Nogo-A-overexpressing cells was reduced progressively due to apoptotic cell death following NGF treatment, but only after 24 h...
January 8, 2018: Cellular Signalling
https://www.readbyqxmd.com/read/29324456/targeted-knockdown-of-bone-morphogenetic-protein-signaling-within-neural-progenitors-protects-the-brain-and-improves-motor-function-following-postnatal-hypoxia-ischemia
#11
Robert W Dettman, Derin Birch, Augusta Fernando, John A Kessler, Maria L V Dizon
Hypoxic-ischemic injury (HI) to the neonatal human brain results in myelin loss that, in some children, can manifest as cerebral palsy. Previously, we had found that neuronal overexpression of the bone morphogenic protein (BMP) inhibitor noggin during development increased oligodendroglia and improved motor function in an experimental model of HI utilizing unilateral common carotid artery ligation followed by hypoxia. As BMPs are known to negatively regulate oligodendroglial fate specification of neural stem cells and alter differentiation of committed oligodendroglia, BMP signaling is likely an important mechanism leading to myelin loss...
January 12, 2018: Developmental Neuroscience
https://www.readbyqxmd.com/read/29323501/the-pivotal-role-of-copper-in-neurodegeneration-a-new-strategy-for-the-therapy-of-neurodegenerative-disorders
#12
Roberta Giampietro, Francesco Spinelli, Marialessandra Contino, Nicola Antonio Colabufo
Copper is an essential trace element for human body since it is a cofactor of several enzymes and proteins and plays a pivotal role in several biological functions (e.g., respiration, protection from oxidative damage, iron metabolism, etc.), also including the central nervous system development and functioning (e.g. synthesis of neurotransmitters, myelination, activation of neuropeptides, etc.). Therefore, copper dysmetabolism is associated with different toxic effects, mainly represented by oxidative stress, and it has been reported in many neurodegenerative disorders, such as Wilson's disease, Menkes disease, Alzheimer's disease, Parkinson's disease, and Amyotrophic Lateral Sclerosis...
January 11, 2018: Molecular Pharmaceutics
https://www.readbyqxmd.com/read/29323104/the-mechanism-of-glycosphingolipid-degradation-revealed-by-a-galc-sapa-complex-structure
#13
Chris H Hill, Georgia M Cook, Samantha J Spratley, Stuart Fawke, Stephen C Graham, Janet E Deane
Sphingolipids are essential components of cellular membranes and defects in their synthesis or degradation cause severe human diseases. The efficient degradation of sphingolipids in the lysosome requires lipid-binding saposin proteins and hydrolytic enzymes. The glycosphingolipid galactocerebroside is the primary lipid component of the myelin sheath and is degraded by the hydrolase β-galactocerebrosidase (GALC). This enzyme requires the saposin SapA for lipid processing and defects in either of these proteins causes a severe neurodegenerative disorder, Krabbe disease...
January 11, 2018: Nature Communications
https://www.readbyqxmd.com/read/29317136/localization-of-the-zinc-binding-tubulin-polymerization-promoting-protein-in-the-mice-and-human-eye
#14
Robert G Tripon, Judit Oláh, Tajwar Nasir, Lajos Csincsik, Chee Lok Li, Sándor Szunyogh, Haiyan Gong, Jane M Flinn, Judit Ovádi, Imre Lengyel
Tubulin Polymerization Promoting Protein (TPPP/p25) modulates the dynamics and stability of the microtubule network by its bundling and acetylation enhancing activities that can be modulated by the binding of zinc to TPPP/p25. Its expression is essential for the differentiation of oligodendrocytes, the major constituents of the myelin sheath, and has been associated with neuronal inclusions. In this paper, evidence is provided for the expression and localization of TPPP/p25 in the zinc-rich retina and in the oligodendrocytes in the optic nerve...
December 27, 2017: Journal of Trace Elements in Medicine and Biology
https://www.readbyqxmd.com/read/29315582/a-dual-role-for-integrin-%C3%AE-6%C3%AE-4-in-modulating-hereditary-neuropathy-with-liability-to-pressure-palsies
#15
Yannick Poitelon, Vittoria Matafora, Nicholas Silvestri, Desirée Zambroni, Claire McGarry, Nora Serghany, Thomas Rush, Domenica Vizzuso, Felipe A Court, Angela Bachi, Lawrence Wrabetz, Maria Laura Feltri
Peripheral myelin protein 22 (PMP22) is a component of compact myelin in the peripheral nervous system. The amount of PMP22 in myelin is tightly regulated, and PMP22 over or under-expression cause Charcot-Marie-Tooth 1A (CMT1A) and Hereditary Neuropathy with Pressure Palsies (HNPP). Despite the importance of PMP22, its function remains largely unknown. It was reported that PMP22 interacts with the β4 subunit of the laminin receptor α6β4 integrin, suggesting that α6β4 integrin and laminins may contribute to the pathogenesis of CMT1A or HNPP...
January 8, 2018: Journal of Neurochemistry
https://www.readbyqxmd.com/read/29314444/a-kinase-anchor-protein-12-is-required-for-oligodendrocyte-differentiation-in-adult-white-matter
#16
Takakuni Maki, Yoon Kyung Choi, Nobukazu Miyamoto, Akihiro Shindo, Anna C Liang, Bum Ju Ahn, Emiri T Mandeville, Seiji Kaji, Kanako Itoh, Ji Hae Seo, Irwin H Gelman, Josephine Lok, Ryosuke Takahashi, Kyu-Won Kim, Eng H Lo, Ken Arai
Oligodendrocyte precursor cells (OPCs) give rise to oligodendrocytes in cerebral white matter. However, the underlying mechanisms that regulate this process remain to be fully defined, especially in adult brains. Recently, it has been suggested that signaling via A-kinase anchor protein 12 (AKAP12), a scaffolding protein that associates with intracellular molecules such as protein kinase A, may be involved in Schwann cell homeostasis and peripheral myelination. Here, we asked whether AKAP12 also regulates the mechanisms of myelination in the CNS...
January 4, 2018: Stem Cells
https://www.readbyqxmd.com/read/29311740/publisher-correction-molecular-mechanisms-of-charcot-marie-tooth-neuropathy-linked-to-mutations-in-human-myelin-protein-p2
#17
Salla Ruskamo, Tuomo Nieminen, Cecilie K Kristiansen, Guro H Vatne, Anne Baumann, Erik I Hallin, Arne Raasakka, Päivi Joensuu, Ulrich Bergmann, Ilpo Vattulainen, Petri Kursula
A correction to this article has been published and is linked from the HTML version of this paper. The error has been fixed in the paper.
January 8, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29302192/identification-of-epigenetically-altered-genes-and-potential-gene-targets-in-melanoma-using-bioinformatic-methods
#18
Honghao Duan, Ke Jiang, Dengke Wei, Lijun Zhang, Deliang Cheng, Min Lv, Yuben Xu, Aimin He
This study aimed to analyze epigenetically and genetically altered genes in melanoma to get a better understanding of the molecular circuitry of melanoma and identify potential gene targets for the treatment of melanoma. The microarray data of GSE31879, including mRNA expression profiles (seven melanoma and four melanocyte samples) and DNA methylation profiles (seven melanoma and five melanocyte samples), were downloaded from the Gene Expression Omnibus database. Differentially expressed genes (DEGs) and differentially methylated positions (DMPs) were screened using the linear models for microarray data (limma) package in melanoma compared with melanocyte samples...
2018: OncoTargets and Therapy
https://www.readbyqxmd.com/read/29302076/candidate-cspg4-mutations-and-induced-pluripotent-stem-cell-modeling-implicate-oligodendrocyte-progenitor-cell-dysfunction-in-familial-schizophrenia
#19
Femke M de Vrij, Christian G Bouwkamp, Nilhan Gunhanlar, Guy Shpak, Bas Lendemeijer, Maarouf Baghdadi, Shreekara Gopalakrishna, Mehrnaz Ghazvini, Tracy M Li, Marialuisa Quadri, Simone Olgiati, Guido J Breedveld, Michiel Coesmans, Edwin Mientjes, Ton de Wit, Frans W Verheijen, H Berna Beverloo, Dan Cohen, Rob M Kok, P Roberto Bakker, Aviva Nijburg, Annet T Spijker, P M Judith Haffmans, Erik Hoencamp, Veerle Bergink, Jacob A Vorstman, Timothy Wu, Loes M Olde Loohuis, Najaf Amin, Carolyn D Langen, Albert Hofman, Witte J Hoogendijk, Cornelia M van Duijn, M Arfan Ikram, Meike W Vernooij, Henning Tiemeier, André G Uitterlinden, Ype Elgersma, Ben Distel, Joost Gribnau, Tonya White, Vincenzo Bonifati, Steven A Kushner
Schizophrenia is highly heritable, yet its underlying pathophysiology remains largely unknown. Among the most well-replicated findings in neurobiological studies of schizophrenia are deficits in myelination and white matter integrity; however, direct etiological genetic and cellular evidence has thus far been lacking. Here, we implement a family-based approach for genetic discovery in schizophrenia combined with functional analysis using induced pluripotent stem cells (iPSCs). We observed familial segregation of two rare missense mutations in Chondroitin Sulfate Proteoglycan 4 (CSPG4) (c...
January 4, 2018: Molecular Psychiatry
https://www.readbyqxmd.com/read/29300891/loss-of-cntnap2-causes-axonal-excitability-deficits-developmental-delay-in-cortical-myelination-and-abnormal-stereotyped-motor-behavior
#20
Ricardo Scott, Alberto Sánchez-Aguilera, Kim van Elst, Lynette Lim, Nathalie Dehorter, Sung Eun Bae, Giorgia Bartolini, Elior Peles, Martien J H Kas, Hilgo Bruining, Oscar Marín
Contactin-associated protein-like 2 (Caspr2) is found at the nodes of Ranvier and has been associated with physiological properties of white matter conductivity. Genetic variation in CNTNAP2, the gene encoding Caspr2, has been linked to several neurodevelopmental conditions, yet pathophysiological effects of CNTNAP2 mutations on axonal physiology and brain myelination are unknown. Here, we have investigated mouse mutants for Cntnap2 and found profound deficiencies in the clustering of Kv1-family potassium channels in the juxtaparanodes of brain myelinated axons...
December 28, 2017: Cerebral Cortex
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