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Fernando Bartolome, Macarena de la Cueva, Consuelo Pascual, Desiree Antequera, Tamara Fernandez, Carmen Gil, Ana Martinez, Eva Carro
BACKGROUND: The phosphodiesterase (PDE) 7 inhibitor S14 is a cell-permeable small heterocyclic molecule that is able to cross the blood-brain barrier. We previously found that intraperitoneal treatment with S14 exerted neuroprotection in an Alzheimer's disease (AD) model (in APP/PS1 mice). The objective of this study was to investigate the neurogenic and cellular effects of oral administration of S14 on amyloid β (Aβ) overload. METHODS: We orally administered the PDE7 inhibitor S14 (15 mg/kg/day) or vehicle in 6-month-old APP/PS1 mice...
February 20, 2018: Alzheimer's Research & Therapy
G Mkrtchyan, A Graf, L Bettendorff, V Bunik
Decreased thiamine and reduced activity of thiamine diphosphate (ThDP)-dependent 2-oxoglutarate dehydrogenase (OGDH) cause neurodegeneration. We hypothesized on concerted cell-specific regulation of the thiamine metabolism and ThDP-dependent reactions. We identified a smaller thiamine pool, a lower expression of the mitochondrial ThDP transporter, and a higher expression of OGDH in rat astrocytes versus neuroblastoma N2A. According to the data, the astrocytic OGDH may be up-regulated by an increase in intracellular ThDP, while the neuroblastomal OGDH functions at full ThDP saturation...
December 2016: Neurochemistry International
Kamaraj Sattu, Falko Hochgräfe, Jianmin Wu, Ganesh Umapathy, Christina Schönherr, Kristina Ruuth, Damini Chand, Barbara Witek, James Fuchs, Pui-Kai Li, Fredrik Hugosson, Roger J Daly, Ruth H Palmer, Bengt Hallberg
Activation of the anaplastic lymphoma kinase (ALK) receptor tyrosine kinase is a key oncogenic mechanism in a growing number of tumor types. In the majority of cases, ALK is activated by fusion with a dimerizing partner protein as a result of chromosomal translocation events, most studied in the case of the nucleophosmin-ALK and echinoderm microtubule-associated protein-like 4-ALK oncoproteins. It is now also appreciated that the full-length ALK receptor can be activated by point mutations and by deletions within the extracellular domain, such as those observed in neuroblastoma...
November 2013: FEBS Journal
Hafsah Kanwal, Mohammad Haroon Khan, Hamid Rashid
Neuroblastoma is a cancer of the sympathetic nervous system, accounting for upto 15% of childhood cancer mortality. It can occur in many areas but most of them begin in the abdomen in the adrenal gland and can spread to the bones and other areas. Unfortunately, like other cancers, its causes are still poorly understood. Anaplastic lymphoma kinase (ALK), a membrane associated tyrosine kinase was recently found to be mutated in neuroblastoma...
May 2013: Pakistan Journal of Pharmaceutical Sciences
Franck Bourdeaut, Sandrine Ferrand, Laurence Brugières, Marjorie Hilbert, Agnès Ribeiro, Ludovic Lacroix, Jean Bénard, Valérie Combaret, Jean Michon, Dominique Valteau-Couanet, Bertrand Isidor, Xavier Rialland, Maryline Poirée, Anne-Sophie Defachelles, Michel Peuchmaur, Gudrun Schleiermacher, Gaëlle Pierron, Marion Gauthier-Villars, Isabelle Janoueix-Lerosey, Olivier Delattre
Neuroblastic tumours may occur in a predisposition context. Two main genes are involved: PHOX2B, observed in familial cases and frequently associated with other neurocristopathies (Ondine's and Hirschsprung's disease); and ALK, mostly in familial tumours. We have assessed the frequency of mutations of these two genes in patients with a presumable higher risk of predisposition. We sequenced both genes in 26 perinatal cases (prebirth and <1 month of age, among which 10 were multifocal), 16 multifocal postnatal (>1 month) cases, 3 pairs of affected relatives and 8 patients with multiple malignancies...
March 2012: European Journal of Human Genetics: EJHG
Pritika J Narayan, Michael Dragunow
There is increasing demand for automated image analysis of cell nuclei to be fast, objective and informative. Here, we have developed a high content analysis method for quantifying histone acetylation within any given population of cells. To demonstrate the utility of this method we quantified the effect of valproic acid (VPA) on histone H3 acetylation levels in SK-N-SH cells, a human neuroblastomal cell line. VPA, commonly used for treatment of bipolar disorder and epilepsy, has also been shown to act as a histone deacetylase inhibitor (HDACi), and to maintain the N-terminals of susceptible histones in an acetylated and transcriptionally active state...
October 30, 2010: Journal of Neuroscience Methods
L de Pontual, S Lyonnet, J Amiel et al.
Biology, genetics and environment of childhood solid tumours set them apart from adult solid tumours. The nature of the progenitor cells from which these tumours arise, and their immature tissue environment, allows childhood solid tumours to develop with fewer defects in cell regulatory processes. Constitutional molecular defects are known to play a role in childhood solid tumours, as shown by the increased incidence of embryonic cancers in children carrying malformations associated with childhood cancer. These rare diagnoses are commonly missed...
August 2010: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
V J Balcar, A Schousboe, P E Spoerri, J R Wolff
High affinity uptake of [(3)H]l-glutamate was studied in cultures of continuous cell lines, originating either from mouse neuroblastoma or rat glioma, and in two types of primary cultures containing cerebellar granule cells and astrocytes from cerebral cortex, respectively. In the continuous lines, d- and l-aspartate-4-hydroxamate were found to interact preferentially with the uptake of [(3)H]l-glutamate in glioma cells while l-glutamate-5-hydroxamate and 2-aminoadipate interacted more strongly with [(3)H]l-glutamate uptake in neuroblastoma cells, d-Aspartate-4-hydroxyamate, l-glutamate-5-hydroxamate and 2-aminoadipate were inactive as inhibitors of [(3)H]l-glutamate uptake by either granule cells or astrocytes, grown in primary culture, but several other glutamate analogues, which did not differentiate between neuroblastomal and gliomal uptake of [(3)H]l-glutamate, were somewhat stronger inhibitors of [(3)H]l-glutamate uptake in astrocytes as compared to that in granule cells...
1987: Neurochemistry International
R Bagna, E Bertino, I Rovelli, C Peila, F Giuliani, L Occhi, M Mensa, R Mazzone, P Saracco, C Fabris
In this case-report a case of severe fetal anemia of unknown origin is presented. Diagnosis of fetal anemia was made at 24 weeks of gestational age, when fetal ascites was identified. Doppler sonography of medium cerebral artery showed a high systolic speed velocity (ACM-PSV), of 65 cm/s (>1.55 MoM). This value predicts a severe fetal anemia. Funicolocentesis confirmed hyporegenerative anemia, low reticulocytosis and low erythroblastosis. A fetal transfusion was performed. At birth anemia was still present and the baby presented blueberry muffin and liver erythropoietic foci...
June 2010: Minerva Pediatrica
C Franzius, M Schmidt, B Hero, T Pfluger, K Hahn
The German translation of the EANM guideline for MIBG scintigraphy in children (Olivier P et al. EJNM MI 2003; 30: B45-B50; Hahn K. Der Nuklearmediziner 2002; 25: 101-105) was reviewed and actualized according to current publications, legal requirements and conditions in Germany. For the first time this guideline was generated in consensus with the neuroblastoma study group of the Association of Paediatric Haematologie and Oncology (GPOH) with the result of an interdisciplinary recommendation. Further main alterations are related to the recommended (123)I activities with respect to the new EANM Paediatric Dosage Card and the explicit recommendation of SPECT...
2008: Nuklearmedizin. Nuclear Medicine
Xiang-ru Wu, Ming-hua Zhu, Zhong-de Zhang, Min-zhi Yin, Zheng-jun Xi, Feng-ying Zhang, Wen-zhu Zhang
OBJECTIVE: To study the expression of E-cadherin and beta-catenin in neuroblastomas of various degrees of differentiation, and to investigate their molecular mechanisms in correlation with clinicopathologic parameters. METHODS: Immunohistochemistry EnVision method was used to detect E-cadherin and beta-catenin expression in 90 paraffin-embedded tissue samples of neuroblastomas. The methylation status of CpG islands of E-cadherin promoter was investigated by MSP in 7 fresh tissue and 24 paraffin-embedded tissue samples...
March 2007: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
W Lux, H-G Klobeck, P B Daniel, M Costa, R L Medcalf, W-D Schleuning
Besides its well-established role in wound healing and fibrinolysis, tissue-type plasminogen activator (t-PA) has been shown to contribute to cognitive processes and memory formation within the central nervous system, and to promote glutamate receptor-mediated excitotoxicity. The t-PA gene is expressed and regulated in neuronal cells but the regulatory transcriptional processes directing this expression are still poorly characterized. We have used DNase I-hypersensitivity mapping and in vivo foot printing to identify putative regulatory elements and transcription factor binding sites in two human neuroblastomal (KELLY and SK-N-SH) and one human glioblastomal (SNB-19) cell lines...
May 2005: Journal of Thrombosis and Haemostasis: JTH
M B Michalowski, H Rubie, J Michon, S Montamat, C Bergeron, C Coze, Y Perel, D Valteau-Couanet, J Guitard, J M Guys, C Piolat, C Munzer, D Plantaz
UNLABELLED: Neuroblastoma is the most frequent tumor observed in the newborn. The aim of this study was to review clinical features, treatment and outcome of newborns diagnosed with a localized neuroblastoma. POPULATION AND METHODS: Data from 52 cases treated according to the NBL 90 and 94 protocols between 1990 and 1999 in 18 French centers of pediatric oncology were analyzed. RESULTS: The median age at diagnosis was 12 days (range 0-28) with antenatal detection in 14 patients (27%)...
July 2004: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
N L'Abbate, M Acquaviva, G de Nichilo, E Paolino, S Pranzo, D Sivo, M G Varraso, C Magnani, G Assennato
(Epidemiological study on childhood tumors and leukaemia) study is an epidemiological case-control study, conducted in 15 Italian regions, including Apulia, to assess the possible risk of childhood cancer (leukaemia, LNH, and Neuroblastomes) resulting from exposures to electromagnetic fields (EMFs).
July 2003: Giornale Italiano di Medicina del Lavoro Ed Ergonomia
Sachiyo Suita
BACKGROUND/PURPOSE: Since 1985, a nationwide mass screening program (MS) for neuroblastoma has been conducted for 6-month-old infants throughout Japan, resulting in the detection of more than 1,900 cases of neuroblastoma. The outcome of these patients has been excellent: more than 97% of them are alive. Yet, several reports suggest that the number of advanced-stage neuroblastoma patients over 1 year of age has not changed substantially. The current report focuses on the 15-year experience with MS of the Kyushu Pediatric Oncology Study Group...
July 2002: Journal of Pediatric Surgery
C Sarkis, C Serguera, S Petres, D Buchet, J L Ridet, L Edelman, J Mallet
Gene delivery to the central nervous system is central to the development of gene therapy for neurological diseases. We developed a baculovirus-derived vector, the Bac-CMV-GFP vector, containing a reporter gene encoding for the green fluorescent protein (GFP) under the control of the cytomegalovirus (CMV) promoter. Two neuroblastomal cell lines and three human primary neural cultures could be efficiently transduced. In all cases, addition of butyrate, an inhibitor of histone deacetylase, increased the level of expression in terms of the number of GFP-expressing cells and the intensity of fluorescence...
December 19, 2000: Proceedings of the National Academy of Sciences of the United States of America
N Keshelava, S Groshen, C P Reynolds
PURPOSE: We have previously shown that neuroblastomal cell lines established from patients after intensive chemotherapy show sustained resistance to various drugs and especially high resistance to etoposide (up to 51 times higher than a clinically achievable level). To determine whether topoisomerase I inhibitors (topotecan and CPT- 11) are effective against etoposide-resistant neuroblastomas, we studied the response to topotecan and the active metabolite of CPT-11 (SN-38) in 19 cell lines with a spectrum of sensitivities to etoposide...
2000: Cancer Chemotherapy and Pharmacology
T Ito, T Hattori, S Okano, T Abe
In a long-term culture of bone marrow taken from a child with a suspected abdominal tumor, a large number of globular in vitro clumps developed within 5 days, although the bone marrow used had tested negative to the presence of abnormal cells. Light microscopic and marker studies of these clumps provided evidence of neuroblastomal origin, and the diagnosis of a neuroblastoma was confirmed by a subsequent cathecholamine assessment and the histological findings of the excised tumor. Of three in vitro culture methods tested, i...
October 1995: International Journal of Hematology
I H Fentie, F J Roisen
Neuro-2a murine neuroblastomal cells exposed to exogenous ganglioside undergo increased neuritogenesis in vitro. To determine if the distribution of exogenous ganglioside (GM1) in neuronal membranes is related to neuritogenesis, the surface topography of exogenous ganglioside in these cells was examined by localization with cholera toxin B-FITC. Following exposure to exogenous ganglioside, levels of fluorescent label appeared similar on perikaryal and neuritic surfaces. Scanning electron microscopic studies using protein G-gold to label antibody against exogenous ganglioside confirmed these observations at higher magnification...
June 1993: Journal of Neurocytology
K Mizuno, S Hashimoto, M Ojima, N Kunii, M Tani, S Niimura, H Watari, S Fukuchi
Readily detectable levels of renin activity were demonstrated in the human brain. This activity was inhibited by specific antibody raised against human renal renin, indicating that it was not due to the nonspecific action of proteases such as cathepsin D. The pineal gland was found to be the richest source of renin followed by the pituitary, hypothalamus and hippocampus. The substantia nigra, caudate nucleus, putamen and thalamus contained moderately high concentrations of renin. The brain renins from pineal and pituitary glands shared some biochemical features with well-known kidney renin, such as molecular weight (46,000 daltons for pineal renin; 37,000-45,000 daltons for pituitary renin), optimum pH (6...
September 1985: Japanese Circulation Journal
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