Abstract:
:The most active corticosteroids are 11 beta-hydroxylated. Humans have two isozymes with 11 beta-hydroxylase activity that are respectively required for cortisol and aldosterone synthesis. CYP11B1 (11 beta-hydroxylase) is expressed at high levels and is regulated by ACTH, whereas CYP11B2 (aldosterone synthase) is normally expressed at low levels and is regulated by angiotensin II. In addition to 11 beta-hydroxylase activity, the latter enzyme has 18-hydroxylase and 18-oxidase activities and thus can synthesize aldosterone from deoxycorticosterone. Insights into the normal functioning of these enzymes are gained from studies of disorders involving them. Mutations in the CYP11B1 gene cause steroid 11 beta-hydroxylase deficiency, a form of congenital adrenal hyperplasia characterized by signs of androgen excess and by hypertension. Mutations in CYP11B2 result in aldosterone synthase (corticosterone methyloxidase) deficiency, an isolated defect in aldosterone biosynthesis that can cause hyponatremia, hyperkalemia, and hypovolemic shock in infancy and failure to thrive in childhood. These are both recessive disorders. Unequal crossing over between the CYP11B genes can generate a duplicated chimeric gene with the transcriptional regulatory region of CYP11B1 but sufficient coding sequences from CYP11B2 so that the encoded enzyme has aldosterone synthase (i.e. 18-oxidase) activity. This results in aldosterone biosynthesis being regulated by ACTH, a condition termed glucocorticoid-suppressible hyperaldosteronism. This form of genetic hypertension is inherited in an autosomal dominant manner.
journal_name
Endocr Revjournal_title
Endocrine reviewsauthors
White PC,Curnow KM,Pascoe Ldoi
10.1210/edrv-15-4-421subject
Has Abstractpub_date
1994-08-01 00:00:00pages
421-38issue
4eissn
0163-769Xissn
1945-7189journal_volume
15pub_type
杂志文章,评审abstract::Rapid effects of steroid hormones result from the actions of specific receptors localized most often to the plasma membrane. Fast-acting membrane-initiated steroid signaling (MISS) leads to the modification of existing proteins and cell behaviors. Rapid steroid-triggered signaling through calcium, amine release, and k...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/er.2007-0022
更新日期:2007-12-01 00:00:00
abstract::Independently, endocrinology, radiology, and nuclear medicine can not optimally differentiate the etiology of the incidental adrenal mass. Rather, the insight necessary for this task must be contributed by all three disciplines. Incidentally discovered adrenal masses are being detected at an increasing rate. This tren...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/edrv-16-4-460
更新日期:1995-08-01 00:00:00
abstract::Polycystic ovary syndrome (PCOS) was hypothesized to result from functional ovarian hyperandrogenism (FOH) due to dysregulation of androgen secretion in 1989-1995. Subsequent studies have supported and amplified this hypothesis. When defined as otherwise unexplained hyperandrogenic oligoanovulation, two-thirds of PCOS...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
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更新日期:2016-10-01 00:00:00
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journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/er.2008-0004
更新日期:2008-10-01 00:00:00
abstract::Steroidogenesis entails processes by which cholesterol is converted to biologically active steroid hormones. Whereas most endocrine texts discuss adrenal, ovarian, testicular, placental, and other steroidogenic processes in a gland-specific fashion, steroidogenesis is better understood as a single process that is repe...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
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更新日期:2011-02-01 00:00:00
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journal_title:Endocrine reviews
pub_type: 杂志文章,评审
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更新日期:2002-12-01 00:00:00
abstract::It is well known that GH is important in the regulation of longitudinal bone growth. Its role in the regulation of bone metabolism in man has not been understood until recently. Several in vivo and in vitro studies have demonstrated that GH is important in the regulation of both bone formation and bone resorption. In ...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/edrv.19.1.0324
更新日期:1998-02-01 00:00:00
abstract::Natriuretic peptides are a family of structurally related but genetically distinct hormones/paracrine factors that regulate blood volume, blood pressure, ventricular hypertrophy, pulmonary hypertension, fat metabolism, and long bone growth. The mammalian members are atrial natriuretic peptide, B-type natriuretic pepti...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
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更新日期:2006-02-01 00:00:00
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journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/er.2003-0003
更新日期:2004-06-01 00:00:00
abstract::This paper has reviewed current knowledge of clinical, biochemical, and morphological manifestations of extracranial GRH-producing tumors. Excessive GRH release stimulates pituitary somatotrophs causing elevation of blood GH levels and acromegaly. In some patients with GRH-containing tumor, blood GH concentrations are...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/edrv-9-3-357
更新日期:1988-08-01 00:00:00
abstract::We applied decision analysis to the controversy over the management of the solitary nonfunctioning thyroid nodule. Three standard management plans were considered: immediate subtotal thyroidectomy; a six month trial of thyroid suppression with L-thyroxine, with non-suppressible lesions being removed surgically; and as...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/edrv-5-2-185
更新日期:1984-04-01 00:00:00
abstract::The secretion of glucocorticoids (GCs) is a classic endocrine response to stress. Despite that, it remains controversial as to what purpose GCs serve at such times. One view, stretching back to the time of Hans Selye, posits that GCs help mediate the ongoing or pending stress response, either via basal levels of GCs p...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/edrv.21.1.0389
更新日期:2000-02-01 00:00:00
abstract::Access to effective affordable contraception is critical for individual and public health. A wide range of hormonal contraceptives (HCs), which differ in composition, concentration of the progestin component, frequency of dosage, and method of administration, is currently available globally. However, the options are r...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/er.2017-00103
更新日期:2018-02-01 00:00:00
abstract::Normal reproductive function in mammals requires precise control of LH synthesis and secretion by gonadotropes of the anterior pituitary. Synthesis of LH requires expression of two genes [alpha-glycoprotein subunit (alphaGSU) and LHbeta] located on different chromosomes. Hormones from the hypothalamus and gonads modul...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/er.2003-0029
更新日期:2004-08-01 00:00:00
abstract::Bile acids derived from cholesterol and oxysterols derived from cholesterol and bile acid synthesis pathways are signaling molecules that regulate cholesterol homeostasis in mammals. Many nuclear receptors play pivotal roles in the regulation of bile acid and cholesterol metabolism. Bile acids activate the farnesoid X...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/er.2000-0035
更新日期:2002-08-01 00:00:00
abstract::Cells from the chorionic girdle of the equine trophoblast invade the maternal endometrium at day 36 of gestation and become established as secretory elements known as the endometrial cups. These structures, which persist for 40-60 days, produce a gonadotropin which can be found in circulation until about day 130 of ge...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/edrv-12-1-27
更新日期:1991-02-01 00:00:00
abstract::Ovarian steroids normally exert homeostatic negative feedback on GnRH release. During sustained exposure to elevated estradiol in the late follicular phase of the reproductive cycle, however, the feedback action of estradiol switches to positive, inducing a surge of GnRH release from the brain, which signals the pitui...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/er.2009-0023
更新日期:2010-08-01 00:00:00
abstract::Thyroid-associated ophthalmopathy (TAO) is a complex disease process presumed to emerge from autoimmunity occurring in the thyroid gland, most frequently in Graves disease (GD). It is disfiguring and potentially blinding, culminating in orbital tissue remodeling and disruption of function of structures adjacent to the...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/er.2018-00066
更新日期:2019-02-01 00:00:00
abstract::Hippo signaling is an evolutionarily conserved pathway that critically regulates development and homeostasis of various tissues in response to a wide range of extracellular and intracellular signals. As an emerging important player in many diseases, the Hippo pathway is also involved in the pathophysiology of diabetes...
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pub_type: 杂志文章,评审
doi:10.1210/er.2017-00167
更新日期:2018-02-01 00:00:00
abstract::In recent years, ovarian tissue cryopreservation has rapidly developed as a successful method for preserving the fertility of girls and young women with cancer or benign conditions requiring gonadotoxic therapy, and is now becoming widely recognized as an effective alternative to oocyte and embryo freezing when not fe...
journal_title:Endocrine reviews
pub_type: 杂志文章
doi:10.1210/endrev/bnaa020
更新日期:2020-12-01 00:00:00
abstract::Insulin degradation is a regulated process that plays a role in controlling insulin action by removing and inactivating the hormone. Abnormalities in insulin clearance and degradation are present in various pathological conditions including type 2 diabetes and obesity and may be important in producing clinical problem...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/edrv.19.5.0349
更新日期:1998-10-01 00:00:00
abstract::Vitamin D deficiency is common in the elderly, especially in the housebound and in geriatric patients. The establishment of strict diagnostic criteria is hampered by differences in assay methods for 25-hydroxyvitamin D. The synthesis of vitamin D3 in the skin under influence of UV light decreases with aging due to ins...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/edrv.22.4.0437
更新日期:2001-08-01 00:00:00
abstract::Normal development and function of the testis are controlled by endocrine and paracrine signaling pathways. Platelet-derived growth factors (PDGFs) are growth factors that mediate epithelial-mesenchymal interactions in various tissues during normal and abnormal processes such as embryo development, wound healing, tiss...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/er.2010-0004
更新日期:2010-12-01 00:00:00
abstract::Pelvic endometriosis is a complex syndrome characterized by an estrogen-dependent chronic inflammatory process that affects primarily pelvic tissues, including the ovaries. It is caused when shed endometrial tissue travels retrograde into the lower abdominal cavity. Endometriosis is the most common cause of chronic pe...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/er.2018-00242
更新日期:2019-08-01 00:00:00
abstract::Disruption of the GH receptor (GHR) gene eliminates GH-induced intracellular signaling and, thus, its biological actions. Therefore, the GHR gene disrupted mouse (GHR-/-) has been and is a valuable tool for helping to define various parameters of GH physiology. Since its creation in 1995, this mouse strain has been us...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
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更新日期:2011-06-01 00:00:00
abstract::Obesity and dyslipidemia are risk factors for metabolic disorders including diabetes and cardiovascular disease. Sphingolipids such as ceramide and glucosylceramides, while being a relatively minor component of the lipid milieu in most tissues, may be among the most pathogenic lipids in the onset of the sequelae assoc...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
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更新日期:2008-06-01 00:00:00
abstract::People with type 2 diabetes are disproportionately affected by cardiovascular disease (CVD), compared with those without diabetes. Traditional risk factors do not fully explain this excess risk, and other "nontraditional" risk factors may be important. This review will highlight the importance of nontraditional risk f...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
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更新日期:2004-02-01 00:00:00
abstract::Estrogens are essential for normal reproductive function. In addition, they exert important, complex, and diverse nonreproductive actions on multiple tissues. Although accumulating evidence from basic science studies using animal models suggests that estradiol plays a critical neuroprotective role against multiple typ...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
doi:10.1210/er.2004-0014
更新日期:2005-05-01 00:00:00
abstract::Spermatogenesis is the process by which a single spermatogonium develops into 256 spermatozoa, one of which will fertilize the ovum. Since the 1950s when the stages of the epithelial cycle were first described, reproductive biologists have been in pursuit of one question: How can a spermatogonium traverse the epitheli...
journal_title:Endocrine reviews
pub_type: 杂志文章,评审
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更新日期:2004-10-01 00:00:00
abstract::There has been an alarming increase in the prevalence of obesity in people with type 1 diabetes in recent years. Although obesity has long been recognized as a major risk factor for the development of type 2 diabetes and a catalyst for complications, much less is known about the role of obesity in the initiation and p...
journal_title:Endocrine reviews
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更新日期:2018-10-01 00:00:00