Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:P61278 (somatostatin)
 22,083 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The present case report demonstrates the history of a 50-year-old man with a mixed endocrine-neurogenous tumor of the ampulla of Vater. The tumor was localized endoscopically after an attack of melena. There were no signs of endocrinopathy. A local resection with suturing of the pancreatic duct was performed. Morphologically, there were two different tissue types (neurogenous and carcinoid-like) with numerous cells and nerve fibers reacting immunohistochemically with somatostatin and neurotensin antisera: some immunoreactivity to PP-antibodies was observed. Still, after 20 months, the patient seems to have been cured by local resection.
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PMID:A metastatic endocrine-neurogenic tumor of the ampulla of Vater with multiple endocrine immunoreaction--malignant paraganglioma? 285 22

Somatostatin (SS) immunoreactivity was localized in a population of neurons in the interpeduncular nucleus (IPN) of the developing and adult cat. SS-immunoreactive perikarya and fibers were found in several subdivisions of the IPN, but were most heavily concentrated in the central nucleus (IPC). Some immunoreactive fibers appeared to be associated with the ventral surface of the IPN where they may come in contact with the interpeduncular cistern. Bundles of immunoreactive fibers also appeared to leave the rostral pole of the IPN bilaterally. Still other SS-positive perikarya and fibers were associated with the tegmentopeduncular tract and the medial division of the dorsal tegmental nucleus. SS was present in both perikarya and fibers of the IPN at birth. The concentration of immunoreactivity increased dramatically during the first 100 days postnatal, but by 180 days the overall intensity of immunoreactivity had decreased to adult levels. These data indicate that SS may be present in the cat IPN in a specific population of neurons that is most active during early postnatal development. The data are consistent with previous suggestions that SS plays a special role in the development of certain neuronal systems and may be particularly important in the integration of behaviors during the neonatal period.
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PMID:The development of somatostatin immunoreactivity in the interpeduncular nucleus of the cat. 286 61

A number of peptide hormones and hormone candidates were studied by immunocytochemistry with respect to their appearance and distribution in the developing porcine gastroenteropancreatic region. The hormones of the pancreatic islets were the first to appear. At 4 weeks' gestation (the earliest stage studied), glucagon, insulin, and somatostatin cells occurred in the dorsal pancreatic primordium, whereas pancreatic polypeptide cells occurred in the ventral primordium. At this stage, the pancreatic primordia were made up of strands of endocrine cells, and no ducts or acini were seen. Subsequently, the endocrine cells were separated by the growing exocrine parenchyma; at still later stages, they aggregated in small nests. Not until birth did they form mantle islets with insulin cells in the central core and the other endocrine cell types on the outside. Gastrin cells appeared in the stomach at the 4-wk stage; somatostatin cells appeared about 1 wk later. In the intestines, these two cell types appeared at the 6-wk stage. Cells displaying glucagon immunoreactivity were the first endocrine cells to appear in the intestine. They occurred in the upper small intestine at the 4-wk stage; they later disappeared from this location but appeared instead in the lower small intestine and colon where they remained. Secretin, cholecystokinin, motilin, gastric inhibitory peptide, and neurotensin cells all appeared at the 6-8-wk stage, and were restricted to the small intestine throughout development. Enkephalin immunoreactive cells appeared late during ontogeny (at the 13-15-wk stage) in both the gut and pancreas. Still later (15-17-wk stage), dense accumulations of endocrine cells (Segi's cap) were occasionally observed on the top of villi in the upper small intestine; these accumulations consisted mainly of somatostatin, cholecystokinin, and gastric inhibitory peptide cells. In view of the early appearance of many gastroenteropancreatic endocrine cell types in fetal life, a functional significance of gastroenteropancreatic hormones in the early development of gut and pancreas is likely.
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PMID:Ontogeny of endocrine cells in porcine gut and pancreas. An immunocytochemical study. 613 93

Intracardiac teratoma is an extremely rare pediatric neoplasm. We studied the case of a 6-year-old girl with a right intraventricular cardiac mass. The tumor consisted of clusters of monotonous round epithelial cells scattered in a dense fibrotic stroma and was thought to represent an atrioventricular nodal tumor. Three years later the tumor recurred, with multiple mature elements derived from all three germ layers, and was diagnosed as mature cystic teratoma. Still present, however, were multiple areas that were histologically similar to the earlier lesion. Immunostaining revealed strong positivity for insulin, glucagon, somatostatin, and chromogranin consistent with overgrown pancreatic islets of Langerhans within a mature teratoma.
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PMID:Intracardiac teratoma in a child simulating an atrioventricular nodal tumor. 785 10

Glucagon is the main counterregulatory hormone in the body. Still, the mechanism involved in the regulation of glucagon secretion from pancreatic alpha cells remains elusive. Dysregulated glucagon secretion is common in patients with Cystic Fibrosis (CF) that develop CF related diabetes (CFRD). CF is caused by a mutation in the Cl- channel Cystic fibrosis transmembrane conductance regulator (CFTR), but whether CFTR is present in human alpha cells and regulate glucagon secretion has not been investigated in detail. Here, both human and mouse alpha cells showed CFTR protein expression, whereas CFTR was absent in somatostatin secreting delta cells. CFTR-current activity induced by cAMP was measured in single alpha cells. Glucagon secretion at different glucose levels and in the presence of forskolin was increased by CFTR-inhibition in human islets, whereas depolarization-induced glucagon secretion was unaffected. CFTR is suggested to mainly regulate the membrane potential through an intrinsic alpha cell effect, as supported by a mathematical model of alpha cell electrophysiology. In conclusion, CFTR channels are present in alpha cells and act as important negative regulators of cAMP-enhanced glucagon secretion through effects on alpha cell membrane potential. Our data support that loss-of-function mutations in CFTR contributes to dysregulated glucagon secretion in CFRD.
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PMID:CFTR is involved in the regulation of glucagon secretion in human and rodent alpha cells. 2827 90

Neurofibromatosis type 1 (NF1) is a hereditary cancer predisposition syndrome characterized by frequent cutaneous and nervous system abnormalities. Patients with NF1 also have an increased prevalence of multiple gastrointestinal and peripancreatic neoplasms-neuroendocrine tumors of the ampulla that express somatostatin are particularly characteristic of NF1. In this study, we characterize the genetic alterations of a clinically well-characterized cohort of six NF1-associated duodenal neuroendocrine tumors using whole-exome sequencing. We identified inactivating somatic mutations in the NF1 gene in three of six tumors; the only other gene altered in more than one tumor was IFNB1. Copy number analysis revealed deletion/loss of heterozygosity of chromosome 22 in three of six patients. Analysis of germline variants revealed germline deleterious NF1 variants in four of six patients, as well as deleterious variants in other tumor suppressor genes in two of four patients with deleterious NF1 variants. Taken together, these data confirm the importance of somatic inactivation of the wild-type NF1 allele in the formation of NF1-associated duodenal neuroendocrine tumors and suggest that loss of chromosome 22 is important in at least a subset of cases. However, we did not identify any genes altered in the majority of NF1-associated duodenal neuroendocrine tumors that uniquely characterize the genomic landscape of this tumor. Still, the genetic alterations in these tumors are distinct from sporadic neuroendocrine tumors occurring at these sites, highlighting that unique genetic alterations drive syndromic tumors.
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PMID:Whole-exome sequencing of duodenal neuroendocrine tumors in patients with neurofibromatosis type 1. 2984 15