Base de dados : MEDLINE
Pesquisa : G04.152.912.750 [Categoria DeCS]
Referências encontradas : 126 [refinar]
Mostrando: 1 .. 10   no formato [Detalhado]

página 1 de 13 ir para página                         

  1 / 126 MEDLINE  
              next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:29489692
[Au] Autor:Hong HN; Shim JH; Won YJ; Yoo JY; Hwang CH
[Ad] Endereço:Department of Anatomy.
[Ti] Título:Therapeutic time window for the effects of erythropoietin on astrogliosis and neurite outgrowth in an in vitro model of spinal cord injury.
[So] Source:Medicine (Baltimore);97(9):e9913, 2018 Mar.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: The objective of this study was to investigate the underlying molecular mechanisms and the therapeutic time window for preventing astrogliosis with erythropoietin (EPO) treatment after in vitro modeled spinal cord injury (SCI). METHODS: Cultured rat spinal cord astrocytes were treated with kainate and scratching to generate an in vitro model of SCI. EPO (100U/mL or 300U/mL) was added immediately or 2, 4, or 8 hours after injury. Some cultures were also treated with AG490, an inhibitor of the EPO-EPO receptor (EpoR) pathway mediator Janus kinase 2 (JAK2). To evaluate neurite extension, rat embryonic spinal cord neurons were seeded onto astrocyte cultures and treated with EPO immediately after injury in the presence or absence of anti-EpoR antibody. RESULTS: EPO treatment at up to 8 hours after injury reduced the expression of axonal growth inhibiting molecules (glial fibrillary acidic protein, vimentin, and chondroitin sulfate proteoglycan), cytoskeletal regulatory proteins (Rho-associated protein kinase and ephephrin A4), and proinflammatory cytokines (tumor necrosis factor-alpha, transforming growth factor-beta, and phosphorylated-Smad3) in a dosedependent manner (P < .001). Most effects peaked with EPO treatment 2-4hours after injury. Additionally, EPO treatment up to 4 hours after injury promoted expression of the EpoR (>2-fold) and JAK2 (>3-fold) in a dose-dependent manner (P < .001), whereas co-treatment with AG490 precluded these effects (P < .001). EPO treatment up to 4hours after injury also enhanced axonal b-III tubulin-immunoreactivity (>12-fold), and this effect was precluded by co-treatment with an anti-EpoR antibody (P < .001). CONCLUSIONS: EPO treatment within 8 hours after injury reduced astrogliosis, and EPO treatment within 4 hours promoted neurite outgrowth. EPO therapy immediately after spinal cord injury may regulate glia to generate an environment permissive of axonal regeneration.
[Mh] Termos MeSH primário: Eritropoetina/administração & dosagem
Gliose/tratamento farmacológico
Crescimento Neuronal/efeitos dos fármacos
Traumatismos da Medula Espinal/tratamento farmacológico
Tempo para o Tratamento
[Mh] Termos MeSH secundário: Animais
Astrócitos
Axônios/efeitos dos fármacos
Células Cultivadas
Esquema de Medicação
Ratos
Ratos Sprague-Dawley
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
11096-26-7 (Erythropoietin)
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180307
[Lr] Data última revisão:
180307
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180301
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009913


  2 / 126 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:29395078
[Au] Autor:Han S; Lee JY; Heo EY; Kwon IK; Yune TY; Youn I
[Ad] Endereço:Biomedical Research Institute, Korea Institute of Science and Technology, 5, Hwarang-ro 14-gil, Seongbuk-gu, Seoul, 02791, Republic of Korea.
[Ti] Título:Implantation of a Matrigel-loaded agarose scaffold promotes functional regeneration of axons after spinal cord injury in rat.
[So] Source:Biochem Biophys Res Commun;496(3):785-791, 2018 02 12.
[Is] ISSN:1090-2104
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:An agarose scaffold can be useful for supporting and guiding injured axons after spinal cord injury (SCI), but the electrophysiological signal of regenerated axon in scaffolds has not yet been determined. The current study investigated whether a Matrigel-loaded agarose scaffold would enhance the regeneration of axons after SCI. Moreover, the functional connectivity of regenerated axons within the channels of the scaffold was evaluated by directly recording motor evoked potentials. Our data showed that the agarose scaffold containing Matrigel can support and enhance linearly organized axon regeneration after SCI. Additionally, motor evoked potentials were successfully recorded from regenerated axons. These results demonstrate that an agarose scaffold loaded with Matrigel could promote the regeneration of axons and guide the reconnection of functional axons after SCI.
[Mh] Termos MeSH primário: Axônios/patologia
Colágeno/química
Regeneração Tecidual Guiada/instrumentação
Laminina/química
Regeneração Nervosa/fisiologia
Proteoglicanas/química
Traumatismos da Medula Espinal/patologia
Traumatismos da Medula Espinal/terapia
Tecidos Suporte
[Mh] Termos MeSH secundário: Animais
Materiais Biomiméticos/síntese química
Combinação de Medicamentos
Desenho de Equipamento
Análise de Falha de Equipamento
Masculino
Crescimento Neuronal
Próteses e Implantes
Ratos
Ratos Sprague-Dawley
Recuperação de Função Fisiológica
Sefarose/química
Traumatismos da Medula Espinal/fisiopatologia
Resultado do Tratamento
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Drug Combinations); 0 (Laminin); 0 (Proteoglycans); 119978-18-6 (matrigel); 9007-34-5 (Collagen); 9012-36-6 (Sepharose)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180222
[Lr] Data última revisão:
180222
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:180204
[St] Status:MEDLINE


  3 / 126 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:29183014
[Au] Autor:Rosso G; Young P; Shahin V
[Ad] Endereço:Institute of Physiology II, WWU Münster, Münster, Germany.
[Ti] Título:Mechanosensitivity of Embryonic Neurites Promotes Their Directional Extension and Schwann Cells Progenitors Migration.
[So] Source:Cell Physiol Biochem;44(4):1263-1270, 2017.
[Is] ISSN:1421-9778
[Cp] País de publicação:Switzerland
[La] Idioma:eng
[Ab] Resumo:BACKGROUND/AIMS: Migration of Schwann cells (SCs) progenitors and neurite outgrowth from embryonic dorsal root ganglions (DRGs) are two central events during the development of the peripheral nervous system (PNS). How these two enthralling events preceding myelination are promoted is of great relevance from basic research and clinical aspects alike. Recent evidence demonstrates that biophysical cues (extracellular matrix stiffness) and biochemical signaling act in concert to regulate PNS myelination. Microenvironment stiffness of SCs progenitors and embryonic neurites dynamically changes during development. METHODS: DRG explants were isolated from day 12.5 to 13.5 mice embryos and plated on laminin-coated substrates with varied stiffness values. After 4 days in culture and immunostaining with specific markers, neurite outgrowth pattern, SCs progenitors migration, and growth cone shape and advance were analyzed with confocal fluorescence microscopy. RESULTS: We found out that growing substrate stiffness promotes directional neurite outgrowth, SCs progenitors migration, growth cone advance and presumably axons fasciculation. CONCLUSIONS: DRG explants are in vitro models for the research of PNS development, myelination and regeneration. Consequently, we conclude the following: Our observations point out the importance of mechanosensitivity for the PNS. At the same time, they prompt the investigation of the important yet unclear links between PNS biomechanics and inherited neuropathies with myelination disorders such as Charcot-Marie-Tooth 1A and hereditary neuropathy with liability to pressure palsies. Finally, they encourage the consideration of mechanosensitivity in bioengineering of scaffolds to aid nerve regeneration after injury.
[Mh] Termos MeSH primário: Neuritos/metabolismo
Crescimento Neuronal/fisiologia
Estresse Mecânico
[Mh] Termos MeSH secundário: Animais
Movimento Celular
Células Cultivadas
Técnicas de Cocultura
Embrião de Mamíferos/citologia
Gânglios Espinais/citologia
Gânglios Espinais/metabolismo
Camundongos
Camundongos Endogâmicos C57BL
Microscopia Confocal
Células de Schwann/citologia
Células de Schwann/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180118
[Lr] Data última revisão:
180118
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171129
[St] Status:MEDLINE
[do] DOI:10.1159/000485485


  4 / 126 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:28957797
[Au] Autor:Tang F; Guo S; Liao H; Yu P; Wang L; Song X; Chen J; Yang Q
[Ti] Título:Resveratrol Enhances Neurite Outgrowth and Synaptogenesis Via Sonic Hedgehog Signaling Following Oxygen-Glucose Deprivation/Reoxygenation Injury.
[So] Source:Cell Physiol Biochem;43(2):852-869, 2017.
[Is] ISSN:1421-9778
[Cp] País de publicação:Switzerland
[La] Idioma:eng
[Ab] Resumo:BACKGROUND/AIMS: Neurite outgrowth and synaptogenesis are critical steps for functional recovery after stroke. Resveratrol promotes neurite outgrowth and synaptogenesis, but the underlying mechanism is not well understood, although the Sonic hedgehog (Shh) signaling pathway may be involved. Given that resveratrol activates sirtuin (Sirt)1, the present study examined whether this is mediated by Shh signaling. METHODS: Primary cortical neuron cultures were pretreated with drugs before oxygen-glucose deprivation/reoxygenation (OGD/R). Cell viability and apoptosis were evaluated with Cell Counting Kit 8 and by terminal deoxynucleotidyl transferase dUTP nick end labeling, respectively. Neurite outgrowth and synaptogenesis were assessed by immunocytochemistry and western blotting, which was also used to examine the expression of Sirt1 and Shh signaling proteins. RESULTS: Resveratrol and the Smoothened (Smo) agonist purmophamine, which activates Shh signaling, increased viability, reduced apoptosis, and stimulated neurite outgrowth after OGD/R injury. Moreover, the expression of growth-associated protein(GAP)-43, synaptophysin, Shh, Patched (Ptc)-1, Smo, glioma-associated oncogene homolog (Gli)-1, and Sirt1 were upregulated under these conditions. These effects were reversed by treatment with the Smo inhibitor cyclopamine, whereas the Sirt1 inhibitor sirtinol reduced the levels of Shh, Ptc-1, Smo, and Gli-1. CONCLUSIONS: Resveratrol reduces neuronal injury following OGD/R injury and enhances neurite outgrowth and synaptogenesis by activating Shh signaling, which in turn induces Sirt1.
[Mh] Termos MeSH primário: Antioxidantes/farmacologia
Glucose/metabolismo
Proteínas Hedgehog/metabolismo
Crescimento Neuronal/efeitos dos fármacos
Fármacos Neuroprotetores/farmacologia
Oxigênio/metabolismo
Estilbenos/farmacologia
[Mh] Termos MeSH secundário: Animais
Sobrevivência Celular/efeitos dos fármacos
Células Cultivadas
Feminino
Neurogênese/efeitos dos fármacos
Neurônios/efeitos dos fármacos
Neurônios/metabolismo
Neurônios/patologia
Ratos Sprague-Dawley
Transdução de Sinais/efeitos dos fármacos
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Antioxidants); 0 (Hedgehog Proteins); 0 (Neuroprotective Agents); 0 (Stilbenes); 0 (sonic hedgehog protein, rat); IY9XDZ35W2 (Glucose); Q369O8926L (resveratrol); S88TT14065 (Oxygen)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171031
[Lr] Data última revisão:
171031
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170929
[St] Status:MEDLINE
[do] DOI:10.1159/000481611


  5 / 126 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:28943240
[Au] Autor:Kempf A; Boda E; Kwok JCF; Fritz R; Grande V; Kaelin AM; Ristic Z; Schmandke A; Schmandke A; Tews B; Fawcett JW; Pertz O; Buffo A; Schwab ME
[Ad] Endereço:Brain Research Institute, University of Zurich and Department of Health Sciences and Technology, Swiss Federal Institute of Technology (ETH) Zurich, 8057 Zurich, Switzerland. Electronic address: anissa.kempf@cncb.ox.ac.uk.
[Ti] Título:Control of Cell Shape, Neurite Outgrowth, and Migration by a Nogo-A/HSPG Interaction.
[So] Source:Dev Cell;43(1):24-34.e5, 2017 Oct 09.
[Is] ISSN:1878-1551
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Heparan sulfate proteoglycans (HSPGs) critically modulate adhesion-, growth-, and migration-related processes. Here, we show that the transmembrane protein, Nogo-A, inhibits neurite outgrowth and cell spreading in neurons and Nogo-A-responsive cell lines via HSPGs. The extracellular, active 180 amino acid Nogo-A region, named Nogo-A-Δ20, binds to heparin and brain-derived heparan sulfate glycosaminoglycans (GAGs) but not to the closely related chondroitin sulfate GAGs. HSPGs are required for Nogo-A-Δ20-induced inhibition of adhesion, cell spreading, and neurite outgrowth, as well as for RhoA activation. Surprisingly, we show that Nogo-A-Δ20 can act via HSPGs independently of its receptor, Sphingosine-1-Phosphate receptor 2 (S1PR2). We thereby identify the HSPG family members syndecan-3 and syndecan-4 as functional receptors for Nogo-A-Δ20. Finally, we show in explant cultures ex vivo that Nogo-A-Δ20 promotes the migration of neuroblasts via HSPGs but not S1PR2.
[Mh] Termos MeSH primário: Movimento Celular/fisiologia
Forma Celular/fisiologia
Proteoglicanas de Heparan Sulfato/metabolismo
Neuritos/metabolismo
Crescimento Neuronal/fisiologia
Proteínas Nogo/metabolismo
[Mh] Termos MeSH secundário: Animais
Proteínas de Transporte/metabolismo
Linhagem Celular
Células Cultivadas
Heparitina Sulfato/metabolismo
Camundongos
Ligação Proteica
Proteoglicanas/metabolismo
Receptores de Lisoesfingolipídeo/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Carrier Proteins); 0 (Heparan Sulfate Proteoglycans); 0 (Nogo Proteins); 0 (Proteoglycans); 0 (Receptors, Lysosphingolipid); 9050-30-0 (Heparitin Sulfate)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171023
[Lr] Data última revisão:
171023
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170926
[St] Status:MEDLINE


  6 / 126 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:28916169
[Au] Autor:Gu T; Zhao T; Kohli U; Hewes RS
[Ad] Endereço:Department of Biology, University of Oklahoma, Norman, OK 73019, USA.
[Ti] Título:The large and small SPEN family proteins stimulate axon outgrowth during neurosecretory cell remodeling in Drosophila.
[So] Source:Dev Biol;431(2):226-238, 2017 11 15.
[Is] ISSN:1095-564X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Split ends (SPEN) is the founding member of a well conserved family of nuclear proteins with critical functions in transcriptional regulation and the post-transcriptional processing and nuclear export of transcripts. In animals, the SPEN proteins fall into two size classes that perform either complementary or antagonistic functions in different cellular contexts. Here, we show that the two Drosophila representatives of this family, SPEN and Spenito (NITO), regulate metamorphic remodeling of the CCAP/bursicon neurosecretory cells. CCAP/bursicon cell-targeted overexpression of SPEN had no effect on the larval morphology or the pruning back of the CCAP/bursicon cell axons at the onset of metamorphosis. During the subsequent outgrowth phase of metamorphic remodeling, overexpression of either SPEN or NITO strongly inhibited axon extension, axon branching, peripheral neuropeptide accumulation, and soma growth. Cell-targeted loss-of-function alleles for both spen and nito caused similar reductions in axon outgrowth, indicating that the absolute levels of SPEN and NITO activity are critical to support the developmental plasticity of these neurons. Although nito RNAi did not affect SPEN protein levels, the phenotypes produced by SPEN overexpression were suppressed by nito RNAi. We propose that SPEN and NITO function additively or synergistically in the CCAP/bursicon neurons to regulate multiple aspects of neurite outgrowth during metamorphic remodeling.
[Mh] Termos MeSH primário: Proteínas de Drosophila/metabolismo
Drosophila melanogaster/metabolismo
Família Multigênica
Crescimento Neuronal
Sistemas Neurossecretores/citologia
Sistemas Neurossecretores/metabolismo
[Mh] Termos MeSH secundário: Animais
Larva/metabolismo
Neurônios/metabolismo
Neurossecreção
Terminações Pré-Sinápticas/metabolismo
Interferência de RNA
Asas de Animais/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T; RESEARCH SUPPORT, U.S. GOV'T, NON-P.H.S.
[Nm] Nome de substância:
0 (Drosophila Proteins)
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171111
[Lr] Data última revisão:
171111
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170917
[St] Status:MEDLINE


  7 / 126 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:28904214
[Au] Autor:Gomez-Sanchez JA; Pilch KS; van der Lans M; Fazal SV; Benito C; Wagstaff LJ; Mirsky R; Jessen KR
[Ad] Endereço:Department of Cell and Developmental Biology, University College London, Gower Street, London WC1E 6BT, United Kingdom.
[Ti] Título:After Nerve Injury, Lineage Tracing Shows That Myelin and Remak Schwann Cells Elongate Extensively and Branch to Form Repair Schwann Cells, Which Shorten Radically on Remyelination.
[So] Source:J Neurosci;37(37):9086-9099, 2017 Sep 13.
[Is] ISSN:1529-2401
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:There is consensus that, distal to peripheral nerve injury, myelin and Remak cells reorganize to form cellular columns, Bungner's bands, which are indispensable for regeneration. However, knowledge of the structure of these regeneration tracks has not advanced for decades and the structure of the cells that form them, denervated or repair Schwann cells, remains obscure. Furthermore, the origin of these cells from myelin and Remak cells and their ability to give rise to myelin cells after regeneration has not been demonstrated directly, although these conversions are believed to be central to nerve repair. Using genetic lineage-tracing and scanning-block face electron microscopy, we show that injury of sciatic nerves from mice of either sex triggers extensive and unexpected Schwann cell elongation and branching to form long, parallel processes. Repair cells are 2- to 3-fold longer than myelin and Remak cells and 7- to 10-fold longer than immature Schwann cells. Remarkably, when repair cells transit back to myelinating cells, they shorten ∼7-fold to generate the typically short internodes of regenerated nerves. The present experiments define novel morphological transitions in injured nerves and show that repair Schwann cells have a cell-type-specific structure that differentiates them from other cells in the Schwann cell lineage. They also provide the first direct evidence using genetic lineage tracing for two basic assumptions in Schwann cell biology: that myelin and Remak cells generate the elongated cells that build Bungner bands in injured nerves and that such cells can transform to myelin cells after regeneration. After injury to peripheral nerves, the myelin and Remak Schwann cells distal to the injury site reorganize and modify their properties to form cells that support the survival of injured neurons, promote axon growth, remove myelin-associated growth inhibitors, and guide regenerating axons to their targets. We show that the generation of these repair-supportive Schwann cells involves an extensive cellular elongation and branching, often to form long, parallel processes. This generates a distinctive repair cell morphology that is favorable for the formation of the regeneration tracks that are essential for nerve repair. Remyelination, conversely, involves a striking cell shortening to form the typical short myelin cells of regenerated nerves. We also provide evidence for direct lineage relationships between: (1) repair cells and myelin and Remak cells of uninjured nerves and (2) remyelinating cells in regenerated nerves.
[Mh] Termos MeSH primário: Bainha de Mielina/metabolismo
Regeneração Nervosa/fisiologia
Crescimento Neuronal
Traumatismos dos Nervos Periféricos/patologia
Traumatismos dos Nervos Periféricos/fisiopatologia
Células de Schwann/patologia
[Mh] Termos MeSH secundário: Animais
Feminino
Masculino
Camundongos
Camundongos Transgênicos
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170929
[Lr] Data última revisão:
170929
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170915
[St] Status:MEDLINE
[do] DOI:10.1523/JNEUROSCI.1453-17.2017


  8 / 126 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:28844905
[Au] Autor:Wang J; Pavlyk I; Vedula P; Sterling S; Leu NA; Dong DW; Kashina A
[Ad] Endereço:University of Pennsylvania, School of Veterinary Medicine, Philadelphia, PA 19104, United States.
[Ti] Título:Arginyltransferase ATE1 is targeted to the neuronal growth cones and regulates neurite outgrowth during brain development.
[So] Source:Dev Biol;430(1):41-51, 2017 10 01.
[Is] ISSN:1095-564X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Arginylation is an emerging protein modification mediated by arginyltransferase ATE1, shown to regulate embryogenesis and actin cytoskeleton, however its functions in different physiological systems are not well understood. Here we analyzed the role of ATE1 in brain development and neuronal growth by producing a conditional mouse knockout with Ate1 deletion in the nervous system driven by Nestin promoter (Nes-Ate1 mice). These mice were weaker than wild type, resulting in low postnatal survival rates, and had abnormalities in the brain that suggested defects in neuronal migration. Cultured Ate1 knockout neurons showed a reduction in the neurite outgrowth and the levels of doublecortin and F-actin in the growth cones. In wild type, ATE1 prominently localized to the growth cones, in addition to the cell bodies. Examination of the Ate1 mRNA sequence reveals the existence of putative zipcode-binding sequences involved in mRNA targeting to the cell periphery and local translation at the growth cones. Fluorescence in situ hybridization showed that Ate1 mRNA localized to the tips of the growth cones, likely due to zipcode-mediated targeting, and this localization coincided with spots of localization of arginylated ß-actin, which disappeared in the presence of protein synthesis inhibitors. We propose that zipcode-mediated co-targeting of Ate1 and ß-actin mRNA leads to localized co-translational arginylation of ß-actin that drives the growth cone migration and neurite outgrowth.
[Mh] Termos MeSH primário: Aminoaciltransferases/metabolismo
Encéfalo/crescimento & desenvolvimento
Encéfalo/metabolismo
Cones de Crescimento/enzimologia
Neuritos/enzimologia
Crescimento Neuronal
[Mh] Termos MeSH secundário: Actinas/metabolismo
Animais
Arginina/metabolismo
Encéfalo/anormalidades
Encéfalo/patologia
Movimento Celular
Camundongos Endogâmicos C57BL
Camundongos Knockout
Camundongos Transgênicos
Proteínas Associadas aos Microtúbulos/metabolismo
Modelos Biológicos
Neuropeptídeos/metabolismo
Biossíntese de Proteínas
RNA Mensageiro/genética
RNA Mensageiro/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, N.I.H., EXTRAMURAL
[Nm] Nome de substância:
0 (Actins); 0 (Microtubule-Associated Proteins); 0 (Neuropeptides); 0 (RNA, Messenger); 0 (doublecortin protein); 94ZLA3W45F (Arginine); EC 2.3.2.- (Aminoacyltransferases); EC 2.3.2.8 (arginyltransferase)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171111
[Lr] Data última revisão:
171111
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170829
[St] Status:MEDLINE


  9 / 126 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:28823707
[Au] Autor:Ivanova EL; Mau-Them FT; Riazuddin S; Kahrizi K; Laugel V; Schaefer E; de Saint Martin A; Runge K; Iqbal Z; Spitz MA; Laura M; Drouot N; Gérard B; Deleuze JF; de Brouwer APM; Razzaq A; Dollfus H; Assir MZ; Nitchké P; Hinckelmann MV; Ropers H; Riazuddin S; Najmabadi H; van Bokhoven H; Chelly J
[Ad] Endereço:Institut de Génétique et de Biologie Moléculaire et Cellulaire, 67400 Illkirch, France; Centre National de la Recherche Scientifique, UMR7104, 67400 Illkirch, France; Institut National de la Santé et de la Recherche Médicale, U964, 67400 Illkirch, France; Université de Strasbourg, 67400 Illkirch, Fr
[Ti] Título:Homozygous Truncating Variants in TBC1D23 Cause Pontocerebellar Hypoplasia and Alter Cortical Development.
[So] Source:Am J Hum Genet;101(3):428-440, 2017 Sep 07.
[Is] ISSN:1537-6605
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Pontocerebellar hypoplasia (PCH) is a heterogeneous group of rare recessive disorders with prenatal onset, characterized by hypoplasia of pons and cerebellum. Mutations in a small number of genes have been reported to cause PCH, and the vast majority of PCH cases are explained by mutations in TSEN54, which encodes a subunit of the tRNA splicing endonuclease complex. Here we report three families with homozygous truncating mutations in TBC1D23 who display moderate to severe intellectual disability and microcephaly. MRI data from available affected subjects revealed PCH, small normally proportioned cerebellum, and corpus callosum anomalies. Furthermore, through in utero electroporation, we show that downregulation of TBC1D23 affects cortical neuron positioning. TBC1D23 is a member of the Tre2-Bub2-Cdc16 (TBC) domain-containing RAB-specific GTPase-activating proteins (TBC/RABGAPs). Members of this protein family negatively regulate RAB proteins and modulate the signaling between RABs and other small GTPases, some of which have a crucial role in the trafficking of intracellular vesicles and are involved in neurological disorders. Here, we demonstrate that dense core vesicles and lysosomal trafficking dynamics are affected in fibroblasts harboring TBC1D23 mutation. We propose that mutations in TBC1D23 are responsible for a form of PCH with small, normally proportioned cerebellum and should be screened in individuals with syndromic pontocereballar hypoplasia.
[Mh] Termos MeSH primário: Doenças Cerebelares/genética
Cerebelo/anormalidades
Proteínas Ativadoras de GTPase/genética
Homozigoto
Microcefalia/genética
Mutação
Malformações do Sistema Nervoso/genética
Neurônios/patologia
[Mh] Termos MeSH secundário: Adolescente
Animais
Células Cultivadas
Doenças Cerebelares/patologia
Cerebelo/patologia
Criança
Pré-Escolar
Deficiências do Desenvolvimento/genética
Deficiências do Desenvolvimento/patologia
Embrião de Mamíferos/metabolismo
Embrião de Mamíferos/patologia
Feminino
Seres Humanos
Deficiência Intelectual/genética
Deficiência Intelectual/patologia
Masculino
Camundongos
Microcefalia/patologia
Malformações do Sistema Nervoso/patologia
Neuroblastoma/genética
Neuroblastoma/patologia
Crescimento Neuronal
Neurônios/metabolismo
Linhagem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (GTPase-Activating Proteins); 0 (Tbc1d23 protein, mouse)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171027
[Lr] Data última revisão:
171027
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170822
[St] Status:MEDLINE


  10 / 126 MEDLINE  
              first record previous record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:28694018
[Au] Autor:Florica RO; Hipolito V; Bautista S; Anvari H; Rapp C; El-Rass S; Asgharian A; Antonescu CN; Killeen MT
[Ad] Endereço:Graduate Program in Molecular Science Program, Ryerson University, 350 Victoria St., Toronto, Ont., Canada M5B 2K3; Dept. of Chemistry and Biology, Ryerson University, 350 Victoria St., Toronto, Ont., Canada M5B 2K3.
[Ti] Título:The ENU-3 protein family members function in the Wnt pathway parallel to UNC-6/Netrin to promote motor neuron axon outgrowth in C. elegans.
[So] Source:Dev Biol;430(1):249-261, 2017 10 01.
[Is] ISSN:1095-564X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:The axons of the DA and DB classes of motor neurons fail to reach the dorsal cord in the absence of the guidance cue UNC-6/Netrin or its receptor UNC-5 in C. elegans. However, the axonal processes usually exit their cell bodies in the ventral cord in the absence of both molecules. Strains lacking functional versions of UNC-6 or UNC-5 have a low level of DA and DB motor neuron axon outgrowth defects. We found that mutations in the genes for all six of the ENU-3 proteins function to enhance the outgrowth defects of the DA and DB axons in strains lacking either UNC-6 or UNC-5. A mutation in the gene for the MIG-14/Wntless protein also enhances defects in a strain lacking either UNC-5 or UNC-6, suggesting that the ENU-3 and Wnt pathways function parallel to the Netrin pathway in directing motor neuron axon outgrowth. Our evidence suggests that the ENU-3 proteins are novel members of the Wnt pathway in nematodes. Five of the six members of the ENU-3 family are predicted to be single-pass trans-membrane proteins. The expression pattern of ENU-3.1 was consistent with plasma membrane localization. One family member, ENU-3.6, lacks the predicted signal peptide and the membrane-spanning domain. In HeLa cells ENU-3.6 had a cytoplasmic localization and caused actin dependent processes to appear. We conclude that the ENU-3 family proteins function in a pathway parallel to the UNC-6/Netrin pathway for motor neuron axon outgrowth, most likely in the Wnt pathway.
[Mh] Termos MeSH primário: Axônios/metabolismo
Proteínas de Caenorhabditis elegans/metabolismo
Caenorhabditis elegans/metabolismo
Proteínas de Membrana/metabolismo
Neurônios Motores/metabolismo
Proteínas do Tecido Nervoso/metabolismo
Crescimento Neuronal
Via de Sinalização Wnt
[Mh] Termos MeSH secundário: Animais
Corpo Celular/metabolismo
Núcleo Celular/metabolismo
Retículo Endoplasmático/metabolismo
Proteínas de Fluorescência Verde/metabolismo
Células HeLa
Seres Humanos
Netrinas
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Caenorhabditis elegans Proteins); 0 (Membrane Proteins); 0 (Nerve Tissue Proteins); 0 (Netrins); 0 (UNC-6 protein, C elegans); 0 (enu-3 protein, C elegans); 147336-22-9 (Green Fluorescent Proteins)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171116
[Lr] Data última revisão:
171116
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170712
[St] Status:MEDLINE



página 1 de 13 ir para página                         
   


Refinar a pesquisa
  Base de dados : MEDLINE Formulário avançado   

    Pesquisar no campo  
1  
2
3
 
           



Search engine: iAH v2.6 powered by WWWISIS

BIREME/OPAS/OMS - Centro Latino-Americano e do Caribe de Informação em Ciências da Saúde