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Tsc and nestin

Le Liang, Hui Leng, Wei Xing, Yanke Guo, Jing Fu, Kangping Ma
OBJECTIVE: To study the clinicopathologic features of tuberous sclerosis complex (TSC). METHODS: The clinicopathologic data of the patients diagnosed as TSC with refractory epilepsy and resection of epileptic focus were retrospectively analyzed. RESULTS: Fourteen cases were included, the mean age was (15.8±12.9) years, with a male predominance (male to female ratio=10:4). Frontal lobe was the most common (13/14) site of involvement. MRI showed multiple patchy long T1 and long T2 signals...
February 2016: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Jonathan M Van Dyke, Ivy M Smit-Oistad, Corey Macrander, Dan Krakora, Michael G Meyer, Masatoshi Suzuki
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by progressive motor dysfunction and loss of large motor neurons in the spinal cord and brain stem. While much research has focused on mechanisms of motor neuron cell death in the spinal cord, degenerative processes in skeletal muscle and neuromuscular junctions (NMJs) are also observed early in disease development. Although recent studies support the potential therapeutic benefits of targeting the skeletal muscle in ALS, relatively little is known about inflammation and glial responses in skeletal muscle and near NMJs, or how these responses contribute to motor neuron survival, neuromuscular innervation, or motor dysfunction in ALS...
March 2016: Experimental Neurology
Joan Liu, Cheryl Reeves, Zuzanna Michalak, Antonietta Coppola, Beate Diehl, Sanjay M Sisodiya, Maria Thom
INTRODUCTION: Activation of the mTOR pathway has been linked to the cytopathology and epileptogenicity of malformations, specifically Focal Cortical Dysplasia (FCD) and Tuberous Sclerosis (TSC). Experimental and clinical trials have shown than mTOR inhibitors have anti-epileptogenic effects in TS. Dysmorphic neurones and balloon cells are hallmarks of FCDIIb and TSC, but similar cells are also occasionally observed in other acquired epileptogenic pathologies, including hippocampal sclerosis (HS) and Rasmussen's encephalitis (RE)...
2014: Acta Neuropathologica Communications
Shawn L Hervey-Jumper, David B Altshuler, Anthony C Wang, Xiaobing He, Cormac O Maher, Patricia L Robertson, Hugh J L Garton, Xing Fan, Karin M Muraszko, Sandra Camelo-Piragua
Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a recently described embryonal neoplasm of the central nervous system, consisting of a well-circumscribed embryonal tumor of infancy with mixed features of ependymoblastoma (multilayer ependymoblastic rosettes and pseudorosettes) and neuroblastoma (neuroblastic rosettes) in the presence of neuropil-like islands. We present the case of a young child with a very aggressive tumor that rapidly recurred after gross total resection, chemotherapy and radiation...
January 2014: Brain Pathology
Vaibhav Saini, Curtis D Hose, Anne Monks, Kunio Nagashima, Bingnan Han, Dianne L Newton, Angelena Millione, Jalpa Shah, Melinda G Hollingshead, Karen M Hite, Mark W Burkett, Rene M Delosh, Thomas E Silvers, Dominic A Scudiero, Robert H Shoemaker
Recently, there has been renewed interest in the role of tumor stem cells (TSCs) in tumorigenesis, chemoresistance, and relapse of malignant tumors including osteosarcoma. The potential exists to improve osteosarcoma treatment through characterization of TSCs and identification of therapeutic targets. Using transcriptome, proteome, immunophenotyping for cell-surface markers, and bioinformatic analyses, heterogeneous expression of previously reported TSC or osteosarcoma markers, such as CD133, nestin, POU5F1 (OCT3/4), NANOG, SOX2, and aldehyde dehydrogenase, among others, was observed in vitro...
2012: PloS One
June Goto, Delia M Talos, Peter Klein, Wei Qin, Yvonne I Chekaluk, Stefanie Anderl, Izabela A Malinowska, Alessia Di Nardo, Roderick T Bronson, Jennifer A Chan, Harry V Vinters, Steven G Kernie, Frances E Jensen, Mustafa Sahin, David J Kwiatkowski
Tuberous sclerosis complex (TSC) is a multiorgan genetic disease in which brain involvement causes epilepsy, intellectual disability, and autism. The hallmark pathological finding in TSC is the cerebral cortical tuber and its unique constituent, giant cells. However, an animal model that replicates giant cells has not yet been described. Here, we report that mosaic induction of Tsc1 loss in neural progenitor cells in Tsc1(cc) Nestin-rtTA(+) TetOp-cre(+) embryos by doxycycline leads to multiple neurological symptoms, including severe epilepsy and premature death...
November 8, 2011: Proceedings of the National Academy of Sciences of the United States of America
Cheng-Chia Yu, Guang-Yuh Chiou, Yi-Yen Lee, Yuh-Lih Chang, Pin-I Huang, Yi-Wei Cheng, Lung-Kuo Tai, Hung-Hai Ku, Shih-Hwa Chiou, Tai-Tong Wong
OBJECTS: Medulloblastoma (MB) is the most malignant primary brain tumor in early childhood that contains cellular and functional heterogeneity. Recent evidence has demonstrated that the tumor stem cells (TSC) may explain the radiochemoresistance of brain tumors, including MB. The aim of the present study is to investigate the possible role of TNF-related apoptosis-inducing ligand (TRAIL) in viability and tumorigenicity of MB cells and MB-derived TSC. METHODS: MB-associated TSC were isolated and cultured by serum-free medium with bFGF and EGF...
July 2010: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Gustavo A Moviglia, Antonio G Carrizo, Gabriela Varela, Carlos A Gaeta, Andrea Paes de Lima, Pablo Farina, Hugo Molina
BACKGROUND: Glioblastoma multiforme (GBM), the most aggressive glioma, presents with a rapid evolution and relapse within the first year, which is attributed to the persistence of tumor stem cells (TSC) and the escape of immune surveillance. Mixed leukocyte culture (MLC) cytoimplant has been shown to function as a powerful intratumor pro-inflammatory cytokine pump. Tumor B-cell hybridoma (TBH) vaccines have been shown to function as antigen-presenting cells. We evaluated the toxicity and efficiency of each treatment alone and in combination...
January 2008: Hematology/oncology and Stem Cell Therapy
Noriyuki Misago, Tetsunori Kimura, Yutaka Narisawa
BACKGROUND: The multiple facial lesions of fibrofolliculoma (FF)/trichodiscoma (TD) and those of fibrous papule (FP; perifollicular fibroma/angiofibroma, AF) are characteristic of Birt-Hogg-Dubé (BHD) syndrome and tuberous sclerosis, respectively. However, there was a recently reported case of BHD syndrome with multiple facial FP lesions and a case of tuberous sclerosis, in which one FF lesion was included among the multiple facial FPs. METHODS: The histopathological and immunohistochemical features of FF/TD and FP lesions were revaluated to study the relationship between the two...
September 2009: Journal of Cutaneous Pathology
Karin Boer, Paul J Lucassen, Wim G M Spliet, Erno Vreugdenhil, Peter C van Rijen, Dirk Troost, Floor E Jansen, Eleonora Aronica
PURPOSE: Focal cortical dysplasia type IIB (FCD IIB) and cortical tubers of patients with tuberous sclerosis complex (TSC) are malformations of cortical development that are frequently associated with intractable epilepsy. Their histopathologic and molecular features suggest developmental abnormalities during the early stages of cortical development, which may involve neural progenitor cells. The aim of our study was to define the expression and cell-specific distribution of doublecortin-like (DCL), a protein critically involved in neuronal division and radial migration during early corticogenesis, in both FCD and TSC...
December 2009: Epilepsia
Amy M McCord, Muhammad Jamal, Uma T Shankavaram, Uma T Shankavarum, Frederick F Lang, Kevin Camphausen, Philip J Tofilon
In vitro investigations of tumor stem-like cells (TSC) isolated from human glioblastoma (GB) surgical specimens have been done primarily at an atmospheric oxygen level of 20%. To determine whether an oxygen level more consistent with in situ conditions affects their stem cell-like characteristics, we compared GB TSCs grown under conditions of 20% and 7% oxygen. Growing CD133(+) cells sorted from three GB neurosphere cultures at 7% O(2) reduced their doubling time and increased the self-renewal potential as reflected by clonogenicity...
April 2009: Molecular Cancer Research: MCR
Hyun-Sik Oh, Min-Cheol Lee, Hyung-Seok Kim, Ji-Shin Lee, Jae-Hyuk Lee, Myeong-Kyu Kim, Young-Jong Woo, Jae-Hyoo Kim, Hyoung-Ihl Kim, Seung-Up Kim
OBJECTS: Balloon cells are histopathological hallmarks of cortical malformations, i.e., focal cortical dysplasia (FCD) of the Taylor type or the cortical tubers of tuberous sclerosis, and they are believed to be the epileptogenic substrate and cause therapeutic drug resistant epilepsy in man. This study was carried out to investigate the developmental histogenesis and epileptogenesis of balloon cells in FCD. MATERIALS AND METHODS: We used an immunohistochemical approach to examine the expressions of primitive neuroepithelial cell antigens (CD34, nestin, and vimentin), ionotrophic glutamate receptor subunits (NR1, NR2A/B, GluR1, GluR2, GluR3, GluR4, and GluR5/6/7), and P-glycoprotein in balloon cells from FCD and normal cerebral cortex epileptogenic lesions...
February 2008: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Yue-Hui Ma, Rolf Mentlein, Friederike Knerlich, Marie-Luise Kruse, H Maximilian Mehdorn, Janka Held-Feindt
According to new hypotheses astrocytomas/gliomas either arise from or attract neural stem cells. Biological markers, particularly antigenic markers, have played a significant role for the characterization of these tumour stem cells (TSCc). Because these studies have been performed with single experimental samples mostly from gliomas, we investigated the expression of the stem cell markers CD133/Prominin, Nestin, Sox-2, Musashi-1, CXCR4, Flt-4/VEGFR-3 and CD105/Endoglin in 72 astrocytomas of different WHO-grades and compared it to normal adult human brain...
January 2008: Journal of Neuro-oncology
D Koji Takahashi, Matthew T Dinday, Nicholas M Barbaro, Scott C Baraban
PURPOSE: In patients with tuberous sclerosis complex (TSC), a wide range of neurologic abnormalities develop, including mental retardation and seizures. Brains from TSC patients are characterized by the presence of cortical tubers, large dysmorphic neurons, and abnormal cytomegalic cells. Although analysis of human TSC brain samples led to the identification of these abnormal cell types, very little is known about how these cells function. In an effort to model TSC-associated CNS abnormalities (and ultimately to analyze the electrophysiologic properties of abnormal cells), we examined Eker rats carrying a Tsc2 mutation...
December 2004: Epilepsia
Y Arai, C A Ackerley, L E Becker
We investigated immunocytochemically the expression of tuberin, the TSC2 gene product, in brain resections from children with and without tuberous sclerosis, to characterize the phenotype of balloon and tumor cells, and to elucidate the relationship between tuberin and formation of subependymal giant-cell tumors. In cortical tubers, tuberin was expressed in processes and cell bodies of balloon cells, which also showed consistent vimentin and nestin immunoreactivity, but no glial fibrillary acidic protein, neurofilament, or galactocerebroside positivity by immunofluorescence confocal microscopy...
September 1999: Acta Neuropathologica
H Yamanouchi, V Jay, J T Rutka, S Takashima, L E Becker
To characterize the giant cells in tuberous sclerosis, we examined immunoreactivity for nestin, vimentin, microtubule-associated protein 1B (MAP1B), MAP2, neurofilament, and glial fibrillary acidic protein (GFAP) in cortical tubers detected in brain specimens from 6 patients with tuberous sclerosis who had undergone surgical resection for treatment of intractable epilepsy. Giant cells with a neuronal appearance, "neuron-like giant cells," had a round centrally-placed nucleus with a single, prominent nucleolus, and Nissl substance was commonly present in cortex...
July 1997: Pediatric Neurology
P B Crino, J Q Trojanowski, M A Dichter, J Eberwine
One hallmark of tuberous sclerosis (TSC) is the presence of highly epileptogenic dysplastic cerebral cortex (tubers) composed of abnormally shaped neurons and giant cells. Mutation of the TSC gene (TSC2) may disrupt differentiation and maturation of neuronal precursors, since the TSC2 gene product tuberin is believed to regulate cellular proliferation. To test the hypothesis that cells in tubers may retain the molecular phenotype of embryonic or immature neurons, tubers from five TSC patients were probed with antibodies to proteins expressed in neuronal precursors (nestin, Ki-67, and proliferating cell nuclear antigen)...
November 26, 1996: Proceedings of the National Academy of Sciences of the United States of America
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