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ependymal cells

Hong Zheng, Wen-Mei Yu, Ronald R Waclaw, Maria I Kontaridis, Benjamin G Neel, Cheng-Kui Qu
Catalytically activating mutations in Ptpn11 , which encodes the protein tyrosine phosphatase SHP2, cause 50% of Noonan syndrome (NS) cases, whereas inactivating mutations in Ptpn11 are responsible for nearly all cases of the similar, but distinct, developmental disorder Noonan syndrome with multiple lentigines (NSML; formerly called LEOPARD syndrome). However, both types of disease mutations are gain-of-function mutations because they cause SHP2 to constitutively adopt an open conformation. We found that the catalytic activity of SHP2 was required for the pathogenic effects of gain-of-function, disease-associated mutations on the development of hydrocephalus in the mouse...
March 20, 2018: Science Signaling
Supawadee Sukseree, Lajos László, Florian Gruber, Sophie Bergmann, Marie Sophie Narzt, Ionela Mariana Nagelreiter, Romana Höftberger, Kinga Molnár, Günther Rauter, Thomas Birngruber, Lionel Larue, Gabor G Kovacs, Erwin Tschachler, Leopold Eckhart
Defects in autophagy and the resulting deposition of protein aggregates have been implicated in aging and neurodegenerative diseases. While gene targeting in the mouse has facilitated the characterization of these processes in different types of neurons, potential roles of autophagy and accumulation of protein substrates in neuroepithelial cells have remained elusive. Here we report that Atg7f/f Tyr-Cre mice, in which autophagy-related 7 (Atg7) is conditionally deleted under the control of the tyrosinase promoter, are a model for accumulations of the autophagy adapter and substrate sequestosome-1/p62 in both neuronal and neuroepithelial cells...
March 17, 2018: Molecular Neurobiology
S Kabatas, C S Demir, E Civelek, I Yilmaz, A Kircelli, C Yilmaz, Y Akyuva, E Karaoz
OBJECTIVE: This study aimed to analyze the effect of human Dental Pulp-Neural Crest Stem Cells (hDP-NCSCs) delivery on lesion site after spinal cord injury (SCI), and to observe the functional recovery after transplantation. METHODS: Neural Crest Stem Cells (NCSCs) were isolated from human Dental Pulp (hDP). The experimental rat population was divided into four groups (n = 6/24). Their behavioral motility was scored regularly. After 4-weeks, rats were sacrificed, and their spinal cords were examined for Green Fluorescent Protein (GFP) labeled hDP-NCSCs by immunofluorescence (IF) staining...
2018: Bratislavské Lekárske Listy
Ellen A G Chernoff, Kazuna Sato, Hai V N Salfity, Deborah A Sarria, Teri Belecky-Adams
The differentiated state of spinal cord ependymal cells in regeneration-competent amphibians varies between a constitutively active state in what is essentially a developing organism, the tadpole of the frog Xenopus laevis , and a quiescent, activatable state in a slowly growing adult salamander Ambystoma mexicanum , the Axolotl. Ependymal cells are epithelial in intact spinal cord of all vertebrates. After transection, body region ependymal epithelium in both Xenopus and the Axolotl disorganizes for regenerative outgrowth (gap replacement)...
2018: Frontiers in Cellular Neuroscience
Marcos Assis Nascimento, Lydia Sorokin, Tatiana Coelho-Sampaio
Fractones are extracellular matrix structures in the neural stem cell niche of the subventricular zone (SVZ), where they appear as round deposits named bulbs or thin branching lines called stems. Their cellular origin and what determines their localization at this site is poorly studied and it remains unclear whether they influence neural stem and progenitor cells formation, proliferation and/or maintenance. To address these questions, we analyzed whole mount preparations of the lateral ventricle of male and female mice by confocal microscopy using different extracellular matrix and cell markers...
March 12, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Ingrid Kratzer, Nathalie Strazielle, Elodie Saudrais, Kati Mönkkönen, Céline Maleval, Sandrine Blondel, Jean-François Ghersi-Egea
Exposure of the developing brain to toxins, drugs, or deleterious endogenous compounds during the perinatal period can trigger alterations in cell division, migration, differentiation, and synaptogenesis, leading to life-long neurological impairment. The brain is protected by cellular barriers acting through multiple mechanisms, some of which are still poorly explored. We used a combination of enzymatic assays, live tissue fluorescence microscopy, and an in vitro cellular model of the blood-CSF barrier to investigate an enzymatic detoxification pathway in the developing male and female rat brain...
March 5, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Yuka Yoshida, Munenori Ide, Hiroya Fujimaki, Nozomi Matsumura, Sumihito Nobusawa, Hayato Ikota, Hideaki Yokoo
A 51-year-old man presented with a 2-week history of malaise. MRI revealed a large solid and cystic lesion with ring enhancement measuring 6.5 cm in diameter in the right frontal lobe. Histologically, the tumor consisted of various components: diffuse growth of atypical astrocytic cells consistent with glioblastoma, fascicular proliferation of atypical spindle cells such as fibrosarcoma, clusters of primitive neuronal cells, and foci of ependymal cells. The sarcomatous component also focally exhibited chondroid and osteoid differentiation...
March 5, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Yonghong Chen, Shujuan Zheng, Luis Tecedor, Beverly L Davidson
Sulfamidase (SGSH) deficiency causes mucopolysaccharidosis type IIIA (MPS IIIA), a lysosomal storage disease (LSD) that affects the CNS. In earlier work in LSD mice and dog models, we exploited the utility of adeno-associated viruses (AAVs) to transduce brain ventricular lining cells (ependyma) for secretion of lysosomal hydrolases into the cerebrospinal fluid (CSF), with subsequent distribution of enzyme throughout the brain resulting in improved cognition and extending lifespan. A critical feature of this approach is efficient secretion of the expressed enzyme from transduced cells, for delivery by CSF to nontransduced cells...
January 31, 2018: Molecular Therapy: the Journal of the American Society of Gene Therapy
Xiwei Shan, Lyl Tomlinson, Qian Yang, Holly Colognato
The regulatory mechanisms that control neural stem cell (NSC) activation in the adult ventricular-subventricular zone (V-SVZ) stem cell niche have been the focus of intense investigation, yet how the niche first develops and organizes is poorly understood. Here, we examined matrix metalloproteinases (MMPs) for potential roles in V-SVZ stem cell niche development. MMP12 was found to promote appropriate niche cellular arrangements, the formation of specialized niche extracellular matrix, and the translational planar cell polarity of ependymal cells that surround and support niche NSCs...
February 28, 2018: Stem Cell Reports
Hrushikesh Kharosekar, Anuj Bhide, Vernon Velho, Sanjay Bijwe
Ependymomas are tumors derived from ependymal cells lining the ventricles or from the central canal of the spinal cord. It usually arises in the ventricles with extra ventricular extension. Less than 15 cases of purely cortical ependymomas are reported. We report a rare case of purely cortical anaplastic ependymoma in a pediatric patient, which is rarely reported.
January 2018: Asian Journal of Neurosurgery
Diana Vidovic, Raul Ayala Davila, Richard M Gronostajski, Tracey J Harvey, Michael Piper
BACKGROUND: Radial glial stem cells within the developing nervous system generate a variety of post-mitotic cells, including neurons and glial cells, as well as the specialised multi-ciliated cells that line the walls of the ventricular system, the ependymal cells. Ependymal cells separate the brain parenchyma from the cerebrospinal fluid and mediate osmotic regulation, the flow of cerebrospinal fluid, and the subsequent dispersion of signalling molecules via the co-ordinated beating of their cilia...
February 16, 2018: Neural Development
Yutaro Hayashi, Sumiko Mikawa, Kazuma Masumoto, Fuminori Katou, Kohji Sato
Growth differentiation factor 11 (GDF11), also known as bone morphogenetic protein 11 (BMP11), is a member of the transforming growth factor β (TGF-β) superfamily. Although GDF11 plays pivotal roles during development, including anterior/posterior patterning, formation of the kidney, stomach, spleen and endocrine pancreas, little information is available for GDF11 expression in the adult central nervous system (CNS). We, thus, investigated GDF11 expression in the adult rat CNS using immunohistochemistry. GDF11 was intensely expressed in most neurons and their axons...
February 12, 2018: Journal of Chemical Neuroanatomy
Mandana Hunter, Nicholas J Demarais, Richard L M Faull, Angus C Grey, Maurice A Curtis
The subventricular zone is a key site of adult neurogenesis and is also implicated in neurodegenerative diseases and brain cancers. In the subventricular zone, cell proliferation, migration and differentiation of nascent stem cells and neuroblasts are regulated at least in part by lipids. The human subventricular zone is distinctly layered and each layer contains discrete cell types that support the processes of neuroblast migration and neurogenesis. We set out to determine the lipid signatures of each subventricular layer in the adult human brain (n = 4)...
February 7, 2018: Scientific Reports
Kristina M Adams Waldorf, Branden R Nelson, Jennifer E Stencel-Baerenwald, Colin Studholme, Raj P Kapur, Blair Armistead, Christie L Walker, Sean Merillat, Jay Vornhagen, Jennifer Tisoncik-Go, Audrey Baldessari, Michelle Coleman, Manjiri K Dighe, Dennis W W Shaw, Justin A Roby, Veronica Santana-Ufret, Erica Boldenow, Junwei Li, Xiaohu Gao, Michael A Davis, Jesica A Swanstrom, Kara Jensen, Douglas G Widman, Ralph S Baric, Joseph T Medwid, Kathryn A Hanley, Jason Ogle, G Michael Gough, Wonsok Lee, Chris English, W McIntyre Durning, Jeff Thiel, Chris Gatenby, Elyse C Dewey, Marian R Fairgrieve, Rebecca D Hodge, Richard F Grant, LaRene Kuller, William B Dobyns, Robert F Hevner, Michael Gale, Lakshmi Rajagopal
Zika virus (ZIKV) is a flavivirus with teratogenic effects on fetal brain, but the spectrum of ZIKV-induced brain injury is unknown, particularly when ultrasound imaging is normal. In a pregnant pigtail macaque (Macaca nemestrina) model of ZIKV infection, we demonstrate that ZIKV-induced injury to fetal brain is substantial, even in the absence of microcephaly, and may be challenging to detect in a clinical setting. A common and subtle injury pattern was identified, including (i) periventricular T2-hyperintense foci and loss of fetal noncortical brain volume, (ii) injury to the ependymal epithelium with underlying gliosis and (iii) loss of late fetal neuronal progenitor cells in the subventricular zone (temporal cortex) and subgranular zone (dentate gyrus, hippocampus) with dysmorphic granule neuron patterning...
February 5, 2018: Nature Medicine
Masahiko Haneda, Shinya Hayashi, Tomoyuki Matsumoto, Shingo Hashimoto, Koji Takayama, Nobuaki Chinzei, Shinsuke Kihara, Kazuhiro Takeuchi, Kotaro Nishida, Ryosuke Kuroda
Inflammation serves an important role in the progression of osteoarthritis (OA), and IL‑1β may act as a catabolic factor on cartilage, reducing the synthesis of primary cartilage components type II collagen and aggrecan. Aquaporin 1 (AQP1) is a 28‑kDa water channel formed of six transmembrane domains on the cell membrane. AQP1 is highly expressed in the anus, gallbladder and liver, and is moderately expressed in the hippocampus, ependymal cells of the central nervous system and articular cartilage. It was hypothesized that AQP1 may be highly expressed in OA cartilage and that it may increase the expression of catabolic factors during inflammatory OA progression...
February 2, 2018: Molecular Medicine Reports
Nagendran Muthusamy, Andrew Brumm, Xuying Zhang, S Thomas Carmichael, H Troy Ghashghaei
The stem cell source of neural and glial progenitors in the periventricular regions of the adult forebrain has remained uncertain and controversial. Using a cell specific genetic approach we rule out Foxj1+ ependymal cells as stem cells participating in neurogenesis and gliogenesis in response to acute injury or stroke in the mouse forebrain. Non stem- and progenitor-like responses of Foxj1+ ependymal cells to injury and stroke remain to be defined and investigated.
January 29, 2018: Scientific Reports
Michael L Hendrickson, Ipshita Zutshi, Alyssa Wield, Ronald E Kalil
There is disagreement in the literature concerning the degree of proliferation of cells in the walls of the third ventricle (3rdV) under normal conditions in the adult mammalian brain. To address this issue, we mapped the cells expressing the neural stem/progenitor cell marker nestin along the entire rostrocaudal extent of the 3rdV in adult male rats and observed a complex distribution. Abundant nestin was present in tanycyte cell bodies and processes, and also was observed in patches of ependymal cells as well as in isolated ependymal cells throughout the walls of the 3rdV...
January 23, 2018: European Journal of Neuroscience
Daniel Garcia-Ovejero, Beatriz Paniagua-Torija, Angel Arevalo-Martin, Beatriz Navarro-Galve, Eduardo Molina-Holgado
In the last few decades many efforts have been dedicated to decipher the nature and regenerative potential of neurogenic niches and endogenous stem cells after damage of the central nervous system. In the spinal cord, it has been largely focused on the ependymal region, which hosts neural precursors/stem cells (NSC) in rodents but differs between species and ages. In the current chapter, we detail our protocol to study the gene expression profile of this region using fresh frozen blocks of rat and human post-mortem spinal cords...
2018: Methods in Molecular Biology
Alexander Baraniskin, Monika Chomiak, Guido Ahle, Thomas Gress, Malte Buchholz, Michael Turewicz, Martin Eisenacher, Michelle Margold, Uwe Schlegel, Wolff Schmiegel, Stephan Hahn, Roland Schroers
Primary lymphomas of the central nervous system (PCNSL) are highly aggressive tumors affecting exclusively the CNS, meninges, and eyes. PCNSL must be separated from secondary spread of systemic lymphoma to the CNS (SCNSL), which may occur at diagnosis or relapse of systemic lymphomas. At present, there are no valid methods to distinguish PCNSL from SCNSL based on tumor biopsy because of similar histological presentation. However, SCNSL and PCNSL are different in terms of prognosis and adequate therapy protocols...
January 11, 2018: Journal of Neuro-oncology
Zakia Abdelhamed, Shawn M Vuong, Lauren Hill, Crystal Shula, Andrew Timms, David Beier, Kenneth Campbell, Francesco T Mangano, Rolf W Stottmann, June Goto
Pediatric hydrocephalus is characterized by an abnormal accumulation of cerebrospinal fluid (CSF) and is one of the most common congenital brain abnormalities. However, little is known about the molecular and cellular mechanisms regulating CSF flow in the developing brain. Through whole-genome sequencing analysis, we report that a homozygous splice site mutation in coiled-coil domain containing 39 (Ccdc39) is responsible for early postnatal hydrocephalus in the progressive hydrocephalus (prh) mouse mutant. Ccdc39 is selectively expressed in embryonic choroid plexus and ependymal cells on the medial wall of the forebrain ventricle, and the protein is localized to the axoneme of motile cilia...
January 9, 2018: Development
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