Abstract:
:Familial glucocorticoid resistance (FGR) is a rare hereditary disorder characterized by hypercortisolism and the absence of stigmata of Cushing's syndrome. The inability of glucocorticoids to exert their effects on target tissues is compensated for by increases in circulating corticotropin (ACTH) and cortisol, the former causing excess secretion of both adrenal androgens and adrenal steroid-biosynthesis intermediates with salt-retaining activity. There is considerable variability in the clinical presentations of FGR ranging from asymptomatic, to isolated chronic fatigue and to hypertension with or without hypokalemic alkalosis or to hyperandrogenism, or both. In women, hyperandrogenism can result in acne, hirsutism, menstrual irregularities, oligoanovulation, and infertility; in men it may lead to infertility and in children to precocious puberty. The reported molecular defects in FGR, such as point mutations and a microdeletion of the glucocorticoid receptor (GR) gene, cause partial resistance by, respectively, compromising the function of the GR or decreasing its intracellular concentration in glucocorticoid target tissues. Complete glucocorticoid resistance is believed to be incompatible with life in humans. Hence, the glucocorticoid resistance cases reported have been partial and of variable degree. The extreme variability in the clinical manifestations of the disorder can, additionally, be explained by differing sensitivity of target tissues to mineralocorticoids or androgens or both, and perhaps by different biochemical defects of the glucocorticoid receptor, causing selective resistance of certain glucocorticoid responses in specific tissues. Isolated tissue-resistance from a somatic mutation of the GR in a corticotropinoma from a patient with Nelson's syndrome was also found, suggesting that this may be a mechanism of tumorigenesis. There is additional evidence that defects of GR function can appear surreptitiously in a variety of clinical conditions, suggesting that glucocorticoid resistance in humans may be involved in the pathogenesis and/or clinical picture of a plethora of disease states, of which FGR is the archetype.
journal_name
Ann N Y Acad Scijournal_title
Annals of the New York Academy of Sciencesauthors
Stratakis CA,Karl M,Schulte HM,Chrousos GPdoi
10.1111/j.1749-6632.1994.tb39257.xsubject
Has Abstractpub_date
1994-11-30 00:00:00pages
362-74; discussion 374-6eissn
0077-8923issn
1749-6632journal_volume
746pub_type
杂志文章,评审abstract::Thalassemia influences individual and family psychosocial functioning. Parental reaction to diagnosis affects the parent-child relationship, parents' roles, and family resources for coping. A child frequently translates parental reactions into personal attitudes of dependency and lowered self-esteem. In coping with on...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
doi:10.1111/j.1749-6632.1990.tb24331.x
更新日期:1990-01-01 00:00:00
abstract::A trial was carried out in alpacas (Lama pacos) and llamas (Lama glama) to determine the economic benefits of controlling both external and internal parasites by the use of ivermectin ("Ivomec," Merck Sharp & Dohme). After four months the treated male alpacas gained on average 3.1 kg more than the untreated males, and...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章
doi:10.1111/j.1749-6632.1992.tb19668.x
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abstract::Most ecological economists believe that the transition to a steady-state economy is necessary to ensure ecological sustainability and to maximize a nation's economic welfare. While some observers agree with the necessity of the steady-state economy, they are nonetheless critical of the suggestion made by ecological ec...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
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abstract::Abnormal function of the lymphatic vessels is associated with a variety of diseases, such as tumor metastasis and lymphedema. The development of strategies for local and controlled induction or inhibition of lymphangiogenesis would thus be of major importance for the treatment of such diseases. Two growth factors, vas...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
doi:10.1111/j.1749-6632.2002.tb04871.x
更新日期:2002-12-01 00:00:00
abstract::5-HT2B receptors, in addition to phospholipase C stimulation, are able to trigger activation of the proto-oncogene product p21ras. During mouse embryogenesis, a peak of 5-HT2B receptor expression is detected at the neurulation stage; we localized the 5-HT2B expression in neural crest cells, heart myocardium, and somit...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
doi:10.1111/j.1749-6632.1998.tb10174.x
更新日期:1998-12-15 00:00:00
abstract::In epithelia and endothelia, overall resistance (TER) is determined by all ion-conductive structures, such as membrane channels, tight junctions, and the intercellular space, whereas the epithelial capacitance is due to the hydrophobic phase of the plasma membrane. Impedance means alternating current resistance and, i...
journal_title:Annals of the New York Academy of Sciences
pub_type: 历史文章,杂志文章,评审
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journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
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更新日期:2009-02-01 00:00:00
abstract::In recent years calcium channel blockers have emerged as a new class of antiarrhythmic agents for the control of certain supraventricular and ventricular arrhythmias. Electrophysiologically, they are heterogeneous but their main action is mediated through a depressant effect on the slow calcium channel in cardiac musc...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
doi:10.1111/j.1749-6632.1988.tb33397.x
更新日期:1988-01-01 00:00:00
abstract::We found a novel genetic abnormality, heterozygous C-->T nucleotide substitution at position -1138 bp in the 5'-flanking region of the fibrinogen A alpha gene, in patients with hypofibrinogenemia. Luciferase reporter assay using the pGL3-basic vector and CHO cells indicates that the transcriptional activity of a vecto...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章
doi:10.1111/j.1749-6632.2001.tb03539.x
更新日期:2001-01-01 00:00:00
abstract::Vaccinia virus encodes secretory proteins termed virokines. One of the major virokines encoded by the N1L open reading frame is the 13.8 kDa protein. A recombinant virus, termed vGK5, lacking this protein when injected intracranially into mice, has one of the highest levels of in vivo attenuation achieved by deletion ...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章
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更新日期:2004-12-01 00:00:00
abstract::(a) The PR elicited by excitotoxic destruction of intrinsic neurons in the CN has a rapid onset, peaks within 2 weeks, and persists indefinitely; (b) the majority of the proliferating cells are not intrinsic to the CNS but are of hematic origin; (c) a small mirror-image response is seen in the contralateral CN; (d) th...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章
doi:10.1111/j.1749-6632.1988.tb27119.x
更新日期:1988-01-01 00:00:00
abstract::Inflammasomes continue to generate interest in an increasing number of disciplines owing to their unique ability to integrate a myriad of signals from pathogen- and damage-associated molecular patterns into a proinflammatory response. This potent caspase-1-dependent process is capable of activating the innate immune s...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
doi:10.1111/nyas.12458
更新日期:2014-06-01 00:00:00
abstract::Thymosin alpha 1, originally isolated as the compound responsible for reconstitution of immune function in thymectomized animal models, has enjoyed a wide-ranging clinical development program over the past decades, extending across multiple companies, indications, countries, and continents. This paper provides an over...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
doi:10.1111/j.1749-6632.2010.05482.x
更新日期:2010-04-01 00:00:00
abstract::In in vitro test systems, chrysotile is markedly toxic, causes chromosomal aberrations, and is capable of inducing morphological and preneoplastic transformation. In carefully designed animal experiments, chrysotile produces lung cancer and mesothelioma as effectively as do the amphiboles tested. Human population stud...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
doi:10.1111/j.1749-6632.1991.tb24496.x
更新日期:1991-12-31 00:00:00
abstract::Synthetic oligodeoxynucleotides (ODN) capable of "neutralizing" or "inhibiting" immune responses have been described. This review will focus on the properties of phosphorothioate ODN that mimic the immunosuppressive activity of the repetitive TTAGGG motifs present in mammalian telomeres. These TTAGGG multimers block t...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
doi:10.1111/j.1749-6632.2009.04970.x
更新日期:2009-09-01 00:00:00
abstract::Potato products contain high amounts of acrylamide, which sometimes exceeds the concentration of 1 mg/L. However, many strategies for acrylamide reduction in potato products are possible. In this work, the different approaches for reducing acrylamide formation have been reviewed, keeping in mind that in the applicatio...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
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更新日期:2008-04-01 00:00:00
abstract::Granulocytic anaplasmosis is a tick-borne, zoonotic disease caused by an obligate intracellular granulocytotropic bacterium, Anaplasma phagocytophilum. A. phagocytophilum and other Anaplasma and Ehrlichia spp. have been variably implicated with an associated hematologic alteration, platelet dysfunction, that can resul...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章
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abstract::In natural tissues, cells form multiple attachment sites to their extracellular matrix. By means of those attachments, cells deform as the tissue deforms in response to the natural mechanical stresses and strains that the tissue must sustain during function. These mechanical forces are the energy input that instruct t...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
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abstract::Reprogramming of energy metabolism is one of the hallmarks of cancer. In normal conditions, cells rely on mitochondrial oxidative phosphorylation to provide energy for cellular activities. Cancer cells are characterized by increased glycolysis and reduced mitochondrial respiratory function. In the past decade, somatic...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
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更新日期:2010-07-01 00:00:00
abstract::Although very few measurements have appeared in the open literature and there seems to be a controversy about the existence of the NDR phenomenon in molecules, the prospects of building such systems have attracted significant attention. In the work reported in this paper we used a model based on DFT calculations of th...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章
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更新日期:2003-12-01 00:00:00
abstract::The following, from the 12th OESO World Conference: Cancers of the Esophagus, includes commentaries on the animal reflux-inflammation models for Barrett's esophagus and esophageal adenocarcinoma; genomic/epigenomic analyses; eflornithine-based combinations; the molecular derangements that promote neoplastic transforma...
journal_title:Annals of the New York Academy of Sciences
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doi:10.1111/nyas.12529
更新日期:2014-09-01 00:00:00
abstract::The mobilization and recruitment of blood and lymphatic vasculatures are widely described in inflammatory bowel diseases (IBDs). Although angiogenesis contributes to intense gut inflammation, it remains unclear whether and when lymphangiogenesis amplifies or protects in IBD. The prolonged maintenance of lymphatic (ove...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
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更新日期:2010-10-01 00:00:00
abstract::The objective of this article is to investigate the influence of endothelin-1 (ET-1) on human monocyte Na(+)/H(+) exchanger (NHE) activity and on the atherosclerosis-related monocyte functions. ET-1 caused an increase in pHi and in (22)Na influx of monocytes. A reversal of ET-1 effect on pHi was observed in the presen...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章
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更新日期:2007-01-01 00:00:00
abstract::It is known that red wines have antioxidant properties owing to the presence of polyphenols and hydroxycinnamates. It has been reported that these compounds are efficient scavengers of peroxynitrite (ONOO(-)). We assayed the ONOO(-)-scavenging activity of plant polyphenols, hydroxycinnamates, and red wines using a flu...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章
doi:10.1111/j.1749-6632.2002.tb02924.x
更新日期:2002-05-01 00:00:00
abstract::1 alpha,25-Dihydroxyvitamin D3, the active form of vitamin D3, induced maturation of circulating monocytes to form macrophage-epithelioid cells and multinucleated giant cells in vitro. Calcitriol not only promoted the differentiation of monocytes, as shown by the marked morphological changes and enhanced secretion of ...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章
doi:10.1111/j.1749-6632.1986.tb18497.x
更新日期:1986-01-01 00:00:00
abstract::A type of unit discharge, termed "luxotonic," has been found in the striate cortex of unanesthetized squirrel monkeys and macaques.6, 21 The firing frequency of these units shows relatively little adaptation, continues indefinitely (hours), and reflects the level of diffuse illumination of the eye. The more numerous "...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章
doi:10.1111/j.1749-6632.1977.tb39723.x
更新日期:1977-01-01 00:00:00
abstract::At concentrations effecting apoptosis, the alkylphosphocholine ErPC3 induced increased expression of the Rb protein in breast cancer (MCF-7) and leukemia (SKW-3, AR-230) cell lines as well as hypophosphorylation (K-562, CMLT-1, DOHH-2) and fragmentation of Rb (BV-173, SKW-3) in leukemia cell lines. ErPC3 exerts at lea...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章
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更新日期:2003-12-01 00:00:00
abstract::Results from two similar experimental systems that attempt to create laboratory geophysical analogs in spherical geometry are presented. In the first system, real time holographic interferometry and shadowgraph visualization are used to study convection in the fluid between two concentric spheres when two distinct buo...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章
doi:10.1111/j.1749-6632.2002.tb05893.x
更新日期:2002-10-01 00:00:00
abstract::Both hereditary and environmental factors influence the risk of cancer. Four risk categories, or oncodemes, can exist for a particular kind of cancer, depending upon the presence of neither, one, or both factors: (1) spontaneous, or background; (2) hereditary; (3) environmental; (4) interactive. In the second, mutatio...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
doi:10.1111/j.1749-6632.1997.tb48593.x
更新日期:1997-12-29 00:00:00
abstract::Gastrointestinal peptides play important roles regulating feeding and energy homeostasis. Most gastrointestinal peptides including glucagon like peptide-1, peptide YY, amylin, and oxytomodulin are anorectic, and only ghrelin is an orexigenic peptide. Ghrelin increases appetite, modulates energy balance, suppresses inf...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
doi:10.1111/j.1749-6632.2010.05509.x
更新日期:2010-07-01 00:00:00