Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a 16-year-old boy with esthesioneuroblastoma that presented with a unilateral tumor extending to the maxillary sinus and periorbital region. Despite initial therapy with gross resection, 5,682 cGy to the tumor bed and chemotherapy, the patient subsequently had a rapid local recurrence with distant metastases. Immunocytochemical, ultrastructural, cytogenetic, and molecular techniques were performed to determine if this tumor was biologically similar to childhood neuroblastoma. Urinary excretion of vanillylmandelic acid (VMA) and homovanillic acid (HVA) were markedly elevated. Chromogranin and neuron specific enolase immunostaining of tumor cells was positive, as seen in neuroblastoma. Electron microscopic studies showed cells that were closely packed and connected by occasional cell junctions. The cell cytoplasm contained moderate amounts of filaments and microtubules. Numerous electron dense granules were observed; however, these granules lacked distinct nucleoids and generally reacted strongly for acid phosphatase, indicating a lysosomal rather than a secretory function. Tumor cells contained near-pseudotetraploid chromosomes, with all chromosomes represented at least three times, and chromosome 5 was present in multiples of eight. Clonal structural abnormalities included 2q+ and 5q+ and multiple double minutes. Northern blot analysis revealed both c-myc and N-myc expression; however, N-myc amplification was not demonstrated, and c-myc expression appeared increased, unlike cases of rapidly progressive neuroblastoma. These results suggest that despite biologic similarities to neuroblastoma in catecholamine excretion and some ultrastructural features, molecular genetic abnormalities differ in this comparatively aggressive case of estesioneuroblastoma.
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PMID:Cytogenetic and molecular evaluation of clinically aggressive esthesioneuroblastoma. 202 81

One hundred and two bone marrow samples were analysed by histological and immunohistochemical methods for neurone specific enolase (NSE). The biopsies were performed to determine the extent of bone marrow disease in 84 neuroblastomas, nine embryonal rhabdomyosarcomas, five Ewing's sarcomas, two cases of Hodgkin's disease and two lymphoblastic lymphomas. Twenty seven (32%) of neuroblastoma bone marrows showed metastases by conventional histological techniques and 33 (39%) after immunohistochemical staining with NSE. Five embryonal rhabdomyosarcomas, five Ewing's sarcomas, and two lymphoblastic lymphomas showed bone marrow metastases. Only one of these cases was reactive for NSE. NSE represents a very sensitive immunomarker for the follow up of neuroblastoma and improves detection of bone marrow invasion by neuroblastoma.
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PMID:Immunohistochemical demonstration of neurone specific enolase in bone marrow infiltrated by neuroblastoma. 203 Jan 50

Twenty-eight malignant olfactory neural tumors representative of the histologic spectrum commonly designated as olfactory neuroblastoma were subdivided into two groups: Group I closely resembling classical neuroblastoma (20 cases), and Group II exhibiting neuroendocrine features (eight cases). Immunohistochemically, the tumors were analyzed by using antibodies to keratin, neurofilament protein, S-100, and neuron specific enolase. Neuron specific enolase was the most consistently positive in both groups. Single S-100 positive cells, within or at the edges of tumor nests, often corresponded ultrastructurally to Schwann cells at the tumor-stroma interface. Keratin and neurofilament proteins were expressed singly or together by a small number of cases in both groups. All 11 tumors examined ultrastructurally exhibited neuronal processes containing dense-core granules. The results indicate the following: (a) the reliable diagnostic utility of electron microscopy; (b) the frequent occurrence of Schwann cells in these tumors despite their inconspicuousness by light microscopy; and (c) the unexpected expression of keratin by tumors in both groups. The single or coexpression of keratin-neurofilament protein may define a subset of these tumors for which the clinical significance is presently unclear.
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PMID:The spectrum of olfactory neural tumors. A light-microscopic immunohistochemical and ultrastructural analysis. 242 66

Several tumor lines of neuronal origin were assayed for the presence of two forms of pp60c-src, designated pp60 and pp60+. To determine the specific kinase activity of pp60 and pp60+, an enzymatic analysis was carried out with neuroblastoma LA-N-5, containing a high level of pp60+, neuroblastoma SK-N-SH, containing a low level of pp60+, fibroblast FSD, containing pp60 but no pp60+, and Rous sarcoma virus transformed 3T3 cells, SR-3T3, containing pp60v-src. Km values for Mg2+-ATP of approximately 21, 8, 17 and 13 microM were determined for pp60src kinase from LA-N-5, SK-N-SH, FSD and SR-3T3 respectively, using enolase as a substrate. The Vmax values for pp60c-src kinase from LA-N-5, SK-N-SH and FSD were similar. The Vmax value of pp60v-src was about 50-fold higher. Thirteen distinct phosphopeptides were found in tryptic digests from pp60 and pp60+ labeled in vivo with [32P]. The presence of one phosphopeptide, derived from the N-terminus, correlated with the level of pp60+ in neuroblastoma cells, but a small amount of this peptide was also detected in fibroblasts. Only phosphoserine was detected in the N-terminus of pp60 and pp60+. Our data strongly suggest that the six extra amino acids, present in pp60+ but not in pp60, do not significantly alter the specific kinase activity of pp60+, but might influence its phosphorylation state.
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PMID:Specific kinase activity and phosphorylation state of pp60c-src from neuroblastomas and fibroblasts. 246 4

We cultured an aspiration fluid of the sternal bone marrow of the patient having adrenal neuroblastoma and established a neuroblastoma cell line (HSNB). The HSNB line has the following biological properties. 1. They are small round in shape and proliferate in flotation while forming cell aggregate, and often they attach the bottom of plastic dish and process the nerve-like fibers. A rough-endoplasmic reticulum are poorly developed, however, a lot of free ribosomes are scattered in the cytoplasm. In the peripheral area of the cells, small spherical secretory granules (60-140 nm in diameter) are existed. One characteristic of this cell is existence of microtubules in the cell-projections. 2. They show a stable growth and the doubling time is about 50 hours. 3. Their chromosome number varied widely and the mode is 46. The double minute chromosomes were present in 50% of cells. 4. When they are transplanted in the cheek pouch of hamster, they produced the neuroblastoma. 5. They produce neuron specific enolase. 6. N-myc gene was amplified ca 250 folds.
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PMID:[Establishment and characterization of human neuroblastoma cell line (HSNB)]. 248 66

The soluble brain protein 14-3-2 first described by Moore and McGregor in 1965 is now known to be a cell specific isoenzyme of the glycolytic enzyme enolase (EC 4.2.1.11), designated neuron specific enolase (NSE). It is not only a marker for all types of neurons, but also for all neuroendocrine or paraneuronal cells. The appearance of NSE is a late event in neural differentiation, thus making NSE a useful index of neural maturation. The demonstration that tumors of the nervous system and of neuroendocrine origin contain NSE has promoted the study of NSE as a possible tumor marker. Immunocytochemistry has been used to identify NSE in cytologic preparations from several types of tumors, offering useful indications for differential diagnosis. NSE levels in serum from tumor patients are not useful in the diagnosis of early stage disease. However, serum NSE levels have been shown to be helpful in the identification of advanced small cell lung cancer, neuroblastoma and several other neoplasms. The main use of serum NSE is the monitoring of chemotherapy and the detection of a relapse in these cases.
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PMID:Clinical biochemistry of neuron specific enolase. 254 72

In view of the personal observation that malignant peripheral neuroectodermal tumours (MPNT) can present different histological growth patterns, 41 cases of MPNT were histologically and immunohistochemically studied. The median age of the 41 patients was 15 years (range: 9 months - 23 years). There were 27 males and 14 females. Most tumours (23/41) were located in the thoracopulmonary region. In 31/41 cases there was bone as well as soft tissue involvement. The following histopathological patterns were found: Ewing's sarcoma-like (n = 7), atypical Ewing's sarcoma-like (n = 4), neuroblastoma-like (n = 8), rhabdomyosarcoma-like (n = 8), and hemangiopericytoma-like (n = 1). In 2 cases combined patterns were noted, one tumour being characterized by neuroblastoma-like and Burkitt's lymphoma-like features. Most cases of MPNT differed from the cytological features of typical Ewing's sarcoma in that they contained hyperchromatic nuclei with distinct nucleoli. Some reticulin fibrils were found in between the cells of some cases. Immunohistochemically, 19/23 cases reacted positively to vimentin, 29/32 to neuron specific enolase (NSE), 16/28 to protein S-100, and 1/9 to glial fibrillary acidic protein. 12/24 cases reacted positively to NSE and protein S-100. Neurofilaments and desmin were not found in the formalin fixed material of the present study. The results show that most cases of MPNT can be distinguished from typical Ewing's sarcoma by cytological and histological findings. Differential diagnosis from atypical Ewing's sarcoma, neuroblastoma, and rhabdomyosarcoma is possible by immunohistochemistry.
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PMID:[Malignant peripheral neuroectodermal tumors. Histological and immunohistological conditions in 41 cases]. 267 76

Growth of the human neuroblastoma IMR-32 in methylcellulose culture was studied. The number of colonies was proportional to the number of seeded cells in all conditions tested: control cultures (CT) and test cultures with epidermal growth factor (EGF), hydrocortisone (HC), combined EGF/HC, fibroblast growth factor (FGF) or nerve growth factor (NGF). A portion of IMR-32 cells formed colonies and all factors were without effect when tested individually. In contrast, the combination of EGF/HC at low cell densities enhanced the number of colonies two-fold as compared to controls. Differentiation in IMR-32 colonies was examined by immunocytochemical detection of cell specific marker proteins. As determined by staining with different markers, at least two cells subpopulations could be established within the same colony. One of them expressed NSE (neuron specific enolase) and was designated as neuronal. The other subpopulation was called non-neuronal since it consisted of vimentin and S-100 protein positive cells which were considerably enhanced in the presence of EGF or EGF/HC. In vitro, the IMR-32 neuroblastoma cell line contains pluripotent stem cells from which are derived distinct phenotypes sensitive to different extrinsic factors. Increasing time in culture enhanced neuronal differentiation. EGF, on the other hand, targeted preferentially the non-neuronal phenotype, and stimulated colony formation and its differentiation.
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PMID:Epidermal growth factor stimulates colony formation and non-neuronal marker protein expression by human neuroblastoma in methylcellulose culture. 269 78

Human cell lines with neuronal and neuroendocrine features were examined for their expression of pp60c-src, the cellular homolog of the transforming gene product pp60v-src of Rous sarcoma virus. Four neuroblastoma (LA-N-5, SH-SY5Y, Paju, and SK-N-MC) and three small-cell lung carcinoma (U-2020, U-1690, and U-1285) cell lines were selected on the basis of their stage of neurocrine differentiation, as determined by the expression of neuron-specific enolase. In an immune complex protein kinase assay, all seven cell lines displayed c-src kinase activity which was considerably higher than that found in nonneurocrine cells (human diploid fibroblasts, glioma, and non-small cell lung carcinoma cell lines). Furthermore, the c-src kinase activity, as determined by autophosphorylation or phosphorylation of an exogenous substrate, enolase, correlated with the stage of neurocrine differentiation. There was an approximately 30-fold difference in c-src kinase autophosphorylation activity between the cell lines representing the highest and lowest stages of neurocrine differentiation. A similar variation was found in the steady-state levels of the c-src protein of these cell lines. Highly differentiated neuroblastoma cells expressed two forms of the src protein. Digestion by Staphylococcus aureus V8 protease did reveal structural diversity in the amino-terminal ends of these c-src molecules. In summary, we found a clear correlation between c-src kinase activity and the stage of neuronal and neuroendocrine differentiation. Thus, the phenotypic similarity between neurons and neuroendocrine cells includes high c-src expression.
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PMID:Expression of c-src in cultured human neuroblastoma and small-cell lung carcinoma cell lines correlates with neurocrine differentiation. 283 Apr 84

Enolase is a vital enzyme of the glycolytic pathway. It exists mainly in two forms, non-neuronal enolase (NNE) and neuron specific enolase (NSE). Neurospora crassa, a filamentous fungus, was used as the source of pure NNE, and by using DEAE-cellulose and a Sephadex G-150 column chromatography highly purified enzyme (20.4 fold purification with 54.7 percent recovery) was obtained. The development profile of the enzyme shows a peak value after 90 hours of mycelial growth from conidia of N. crassa. In this respect, it differs from neuroblastoma NSE where the peak value of the enzyme activity appears 7 1/2 hours after the splitting of the cells. N. crassa enolase (NNE) is more thermolabile than NG108 NSE and N. crassa enolase is more sensitive to urea, chloride, and fluorophosphate. The Km values for 2-phosphoglycerate and Mg++ were 0.34 mM and 0.47 mM, respectively, for N. crassa enolase, whereas these values were 1.1 mM and 3.1 mM, respectively, in the case of neuroblastoma NSE. N. crassa enolase is a dimer molecule of molecular weight 85,000 daltons. N. crassa enolase is not neutralized by NSE antisera and neutralized by NNE antisera as opposed to neuroblastoma NSE.
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PMID:Isolation and characterization of non-neuronal enolase (NNE) from Neurospora crassa and comparison with neuron specific enolase isolated from neuroblastoma cell line NG108. 296 42


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