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

We have found specific receptors for atrial natriuretic factor (ANF) in cultured neuroblastoma cells (N4TG1) of peripheral ganglionic origin. Scatchard analysis of the displacement binding revealed noninteracting, single-class binding sites with a KD of 1 X 10(-10) M and a density (Bmax) of 110,000-150,000 sites/cell. The cell-bound 125I-ANF was displaced by unlabeled ANF in a dose-dependent manner. Hormones unrelated to ANF such as angiotensins, adrenocorticotropic hormone, or arginine vasopressin were ineffective in displacing the cell-bound radioactivity. Using azidobenzoyl-125I-ANF as a photoaffinity ligand, an ANF receptor with an apparent Mr of 138,000 was identified by sodium dodecyl sulfate-polyacrylamide gel electrophoresis and autoradiography. The addition of unlabeled ANF (1 microM) to the incubation medium completely abolished the labeling of this protein band, but atriopeptin I (1 microM) or angiotensins I, II, and III (each 1 microM) were not effective in inhibiting the affinity labeling. The treatment of the neuroblastoma cells with ANF stimulated intracellular cyclic GMP levels in a dose-dependent manner with an EC50 of 5 nM. ANF (1 X 10(-7) M) stimulated cyclic GMP accumulation in less than 5 min by 30-fold as compared to the controls.
 ...
PMID:Identification of atrial natriuretic factor receptor of neuroblastoma N4TG1 cells: binding characteristics and photoaffinity labeling. 303 Dec 16

The syndrome of opsoclonus and myoclonus may be the first presenting symptom of neuroblastoma. The disorder is often controlled by treatment with adrenocorticotropic hormone (ACTH). A child with this disorder and treated with ACTH gel had abnormal uptake of 67Ga in both adrenal glands during studies to attempt to detect an occult neuroblastoma. Repeat 67Ga scans proved to be normal once the ACTH was discontinued and the patient was treated with prednisone. It is concluded that ACTH stimulation of normal adrenal tissue was responsible for these abnormal findings.
 ...
PMID:Positive gallium scan in the syndrome of opsoclonus-myoclonus treated with adrenocorticotropic hormone. 633 27

Nine human neuroblastomas were serially transplanted in nude mice. All transplanted tumors were taken from patients older than the age of 1 year 10 months. In contrast, the authors were unable to transplant tumors from patients younger than 1 year 7 months of age, in spite of many attempts. All transplanted tumors produced varying amounts of cathecolamines as revealed by the liquid chromatography and formalin-induced fluorescence techniques. The content of the cathecolamines was closely correlated with the number and size of secretory granules. In high cathecolamine-producing tumors, cell clusters showing strong yellow-orange fluorescence plus a positive argyrophil reaction suggesting pheochromoblastic differentiation were demonstrated by our combined cathecolamine fluorescence and argyrophil reaction method. One of the transplantable tumor lines was demonstrated by unique host responses and biochemical data, to produce adrenocorticotropic hormone and increased cathecolamines. Our observations indicate that neuroblastoma derived from neural crest cells seem to have a nearly complete hormone production function as compared with their normal counterparts.
 ...
PMID:Morphology and function of human neuroblastoma xenotransplanted in nude mice. 671 47

We reviewed the neurologic and developmental courses in 10 children with opsoclonus-myoclonus ("dancing eyes syndrome") and neuroblastoma. All patients are alive without evidence of neoplastic disease after 8+ to 111+ months of follow-up. All had localized disease and 50% had extraabdominal tumors. Neuroblastomas of nine children had favorable Shimada histologic characteristics, and all tumors had single copies of the N-myc oncogene. After neuroblastoma resection, all patients had persistent opsoclonus-myoclonus or ataxia that responded to therapy with adrenocorticotropic hormone. Nine children had relapses of neurologic symptoms. Three years after resection, six of seven patients with sufficient follow-up were free of symptoms and had discontinued therapy. However, nine children had chronic neurologic deficits, including cognitive and motor delays, language deficits, and behavioral abnormalities. All six patients in educational programs required special assistance. Five children required physical, occupational, or speech therapy. Long-term developmental and cognitive problems should be anticipated in patients with neuroblastoma who have opsoclonus-myoclonus or ataxia or both, and early intervention should be instituted to try to minimize these deficits.
 ...
PMID:Long-term outcome in children with opsoclonus-myoclonus and ataxia and coincident neuroblastoma. 775 22

Myoclonic encephalopathy of infancy (MEI) is a unique cause of acute ataxia in infants and is a rare presentation of neuroblastoma. Five cases presenting to a tertiary referral children's hospital during a 10 year period are reviewed. Two cases were associated with a neuroblastoma. All children were treated with intramuscular injections of adrenocorticotropic hormone, with symptomatic improvement. One child died from an opportunistic infection following chemotherapy for neuroblastoma. The four survivors have mild to moderate clinical and intellectual deficits. Investigation and continuing observation for occult neural crest tumours is emphasized for all cases of MEI, though no underlying cause was found in 60% of children in this study.
 ...
PMID:Myoclonic encephalopathy of infancy: a 10 year review. 786 61

A case of olfactory neuroblastoma in a 36-year-old woman who presented with florid Cushing's syndrome is reported. A nasal polyp, which proved to be an olfactory neuroblastoma, was resected. The procedure was followed by complete remission from the endocrinologic abnormalities. Postoperatively, the patient was well for 5 years until recurrence of both Cushing's syndrome and the nasal polyp was noted. Following combined transnasal-transcranial resection of the tumor, which extended into the anterior cranial fossa, the patient again experienced complete remission of Cushing's syndrome. Immunohistochemistry showed the tumor to be positive for neuron-specific enolase, synaptophysin, chromogranin, adrenocorticotropic hormone, beta-endorphin, and S-100 protein. Electron microscopy revealed neuritic processes containing microtubules and neurosecretory granules. This is the first reported case of Cushing's syndrome secondary to olfactory neuroblastoma.
 ...
PMID:Cushing's syndrome secondary to olfactory neuroblastoma. 819 48

We previously reported that polymer-encapsulated mouse neuroblastoma cells that are capable of secreting beta-endorphin may reduce pain sensitivity in rats after capsule implantation into the cerebrospinal fluid (CSF)-filled subarachnoid space of the spinal cord. The neuroblastoma cells carry the proopiomelanocortin (POMC) gene that encodes the precursor of adrenocorticotropic hormone (ACTH) and beta-endorphin. To control the expression of these hormones in the present study, a promoter that is inducible by administration of tetracycline derivatives such as doxycycline (Dox) was linked to the POMC gene. Encapsulated cells in the CSF space of rats stimulated by four intraperitoneal doses of Dox responded with ACTH expression as determined in a subsequence 36-hr in vitro incubation. The amount of ACTH released was dependent on the in vivo Dox dose. These findings indicate that gene expression in xenogeneic cells in the CSF space can be manipulated by injection of a relatively innocuous drug, and suggest that this system may be applicable to cell transplantation therapy in patients with central nervous system diseases that require temporary control of ligand delivery.
 ...
PMID:Dose-dependent doxycycline-mediated adrenocorticotropic hormone secretion from encapsulated Tet-on proopiomelanocortin Neuro2A cells in the subarachnoid space. 960 8

We describe the case of a 2-year-old girl with opsoclonus-myoclonus syndrome treated with chronic adrenocorticotropic hormone (ACTH) in which a metaiodobenzylguanidine scan showed abnormal radiotracer uptake in the left adrenal gland region, interpreted as the site of an occult neuroblastoma. As this finding was not corroborated by previous or subsequent metaiodobenzylguanidine scans or by computed tomography (CT) or magnetic resonance imaging (MRI), we attribute the finding to being a false-positive result from adrenal hyperplasia owing to chronic use of ACTH and not to neuroblastoma. Metaiodobenzylguanidine scintigraphy is an extremely important nuclear medicine examination tool used for the evaluation and staging of pediatric neuroblastoma. We highlight the need for cautious interpretation of metaiodobenzylguanidine as a screening tool for neuroblastoma in patients treated with ACTH.
 ...
PMID:False-positive metaiodobenzylguanidine scan for neuroblastoma in a child with opsoclonus-myoclonus syndrome treated with adrenocorticotropic hormone (acth). 1697 Aug 54

Classically, upon hypothalamic stimulation, adrenocorticotropic hormone (ACTH) is released from the pituitary and acts on melanocortin 2 receptors (MC2R) in the adrenal cortex, stimulating glucocorticoid synthesis and release. Our earlier studies suggested that ACTH might have a direct effect on sympathetic ganglia. To analyze further the involvement of ACTH in regulation of gene expression of norepinephrine (NE) biosynthetic enzymes, we examined the effect of bilateral adrenalectomy (ADX) of Sprague-Dawley male rats. Fourteen days post-ADX, as expected, plasma ACTH was elevated, and levels of tyrosine hydroxylase (TH), dopamine beta-hydroxylase (DBH) and MC2R mRNAs in superior cervical ganglia (SCG), and TH mRNA in locus coeruleus (LC) were increased compared with sham-operated animals. To determine effect of pulsatile elevation of ACTH, corticosterone pellets were implanted to ADX rats. Similar to immobilization (IMO) stress ACTH injections to these animals caused a rise in ACTH in plasma and triggered elevation of TH and DBH mRNAs in SCG and in LC with single and repeated daily injections, and MC2R mRNA in SCG with single injections. To study the effect of ACTH in isolated cells, primary cultures of rat SCG were transfected with TH and DBH promoter constructs and treated with ACTH. In agreement with the in vivo data, ACTH elevated their promoter activities similar to levels triggered by cyclic AMP analog. ACTH in the human SK-N-SH neuroblastoma cells increased TH and DBH promoter activity and endogenous DBH mRNA levels. The results show that ACTH can have a direct effect on transcription and gene expression of NE biosynthetic enzymes even without contribution of adrenal hormones.
 ...
PMID:Adrenocorticotropic hormone elevates gene expression for catecholamine biosynthesis in rat superior cervical ganglia and locus coeruleus by an adrenal independent mechanism. 1844 Jul 7

Olfactory neuroblastomas are rare, slow-growing malignant tumors, usually diagnosed at advanced stages. Ectopic adrenocorticotropic hormone (ACTH) syndrome caused by an olfactory neuroblastoma is extremely rare. We reported two Korean women who suffered from ectopic ACTH syndrome (EAS) caused by olfactory neuroblastomas. The first patient was a 66-year-old woman who had been diagnosed as olfactory neuroblastoma and refused the management two years before and the second patient was a 37-year-old woman on chemotherapy for olfactory neuroblastoma. In the first case, she presented the Cushingoid appearance with systemic edema and her tumor was removed surgically. ACTH secretion by the tissue was confirmed by immunohistochemistry. By contrast, the second patient presented as severe pneumonia caused by cytomegalovirus and was treated with anti-viral agent followed by chemotherapy and radiotherapy, and her residual mass remained. However, after treatment, both patients' plasma ACTH and cortisol levels returned to normal without any adrenolytic therapy. Considering the causative tumors of EAS can be rarely cured and EAS increases the susceptibility to infections, it is prudent to suppress any hypercortisolemia initially, apart from treating the causal malignancy.
 ...
PMID:Two cases of ectopic adrenocorticotropic hormone syndrome with olfactory neuroblastoma and literature review. 1846 86


1 2 Next >>