Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01189 (beta-endorphin)
 21,003 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 24-year-old man visited our hospital complaining of hypertension and headache. Endocrinological findings revealed no abnormalities except for a slight decrease in serum adrenocorticotropic hormone (ACTH), a slight increase in urine 17-ketosteroid (17-KS), and a marked increase in serum pregnenolone. Computed tomography and magnetic resonance imaging revealed a 3 x 3 cm mass in the right adrenal area and I131-aldosterol scintigraphy demonstrated a high absorption of the isotope in the right adrenal area. Vena cavography suggested a 2 x 2 cm tumor thrombus originating in the right adrenal. Under the diagnosis of the right adrenocortical carcinoma, adrenalectomy and removal of the tumor thrombus were performed. Both serum pregnenolone and urine 17-KS returned to the normal level within a week after the operation and blood pressure was well controlled without any medication 3 months after the operation. Thus, the tumor seemed to be endocrinologically active.
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PMID:[Adrenocortical carcinoma: report of a case]. 164 74

From January 1962 through April 1988, 188 patients with Cushing's syndrome were hospitalized at the Veterans General Hospital. Iatrogenic Cushing's syndrome was found in 123 (65.4%) of the patients. The other 43 patients (22.9%) had excess secretion of adrenocorticotropin (ACTH) and 22 patients (11.7%) had benign or malignant adrenocortical tumors. The clinical features and the functional reserve of the adrenal glands in those patients with iatrogenic Cushing's syndrome were evaluated. A retrospective analysis of the diagnostic rate of the various endocrine evaluations and the radiological examinations was also carried out. Four approaches were used in the management of Cushing's syndrome: (1) pituitary surgery, (2) pituitary irradiation, (3) adrenal surgery, and (4) drug therapy. Both the clinical and hormonal responses to these four management types were evaluated. The endocrinological, radiological, surgical and pathological experiences of 25 patients with Cushing's disease treated with pituitary surgery are presented. Five patients (20%) had an atypical endocrine pattern (20% false-negative). Twenty-four patients received a pituitary CT scan and 16 patients (66.7%) had positive CT findings. Of these 25 patients, 19 had pathology-proven microadenomas (less than 1cm), one had macroadenoma (greater than 1cm) and 5 had no evidence of adenomatous tissue. Among the 22 patients with adrenal Cushing's syndrome, 4 (18%) were suffering from adrenocortical carcinoma and 18 (82%) with benign adrenal tumors. The incidence of left adrenal adenoma was approximately twice that of right adrenal adenoma. The cure rate of adrenocortical adenoma by surgical treatment with unilateral adrenalectomy was excellent, but for adrenocortical carcinoma was poor.
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PMID:Cushing's syndrome: analysis of 188 cases. 262 29

A 36-year-old woman who had experienced two pregnancies consulted our hospital, because of scant menses and virilization. A 24-hour excretion of 17-ketosteroids and 17-hydroxy-corticosteroids demonstrated a decrease in 11-hydroxylase. A computed tomogram showed a huge inhomogenous tumefaction in the left adrenal. Left selective renal angiography revealed a large adrenal tumefaction. Selective adrenal venous samplings revealed that testosterone and dehydroepiandrosterone (DHA) were produced in response to stimulation by 0.25 mg exogenous adrenocorticotropic hormone (ACTH). After left adrenalectomy was performed, a diagnosis of adrenocortical carcinoma was made by pathological examination. This is the first report of a patient with a virilizing adrenocortical carcinoma, which produced testosterone and DHA in response to exogenous ACTH stimulation.
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PMID:A case of virilizing adrenocortical carcinoma. 283 27

Very little has been known of the biochemical function of a human adrenocortical carcinoma cell line, SW-13. In this study, the production of several adrenal steroids and 3', 5'-cyclic adenosine monophosphate (cAMP) were investigated in this cell line. The cells were incubated in L-15 medium containing 0.1% bovine serum albumin with several reagents in an atmosphere of 5% CO2 and 95% air for 2 hours at 37 degrees C. Aldosterone (Ald), corticosterone (B), cortisol (F), dehydroepiandrosterone sulfate (DHEA-S) and cAMP were simultaneously assayed by specific radioimmunoassays in the medium and cells. Significant increases in cAMP production were observed by cholera toxin (10 ng/ml) and forskolin (10 nM), both direct stimulators of adenylate cyclase, in the cAMP concentration without an increase in the steroids. The DHEA-S concentration in the medium was significantly increased by angiotensin-II (10(-7)M), noradrenalin (3 X 10(-5) M), adrenalin (3 X 10(-5) M) or alpha-melanocyte-stimulating hormone (alpha-MSH, 10(-7) M), none of which was associated with cAMP production. Neither adrenocorticotropin (10(-10) M) nor human chorionic gonadotropin (500 mIU/ml) stimulated the release of the steroids or cAMP production. A calcium ionophore, A23187 (10(-7) M), and 12-O-tetradecanoylphorbol-13-acetate (10(-8) M), a direct stimulator of protein kinase C, stimulated the release of DHEA-S, but not those of Ald, B and F. The results suggest that SW-13 retains functioning adenylate cyclase which, however, is not linked with steroidogenesis and that DHEA-S is produced probably by the mechanisms which involve protein kinase C system or calcium ion. This report provides the first demonstration of cAMP and DHEA-S production in SW-13 and suggests that this cell line is potentially useful for investigating the mechanisms of steroidogenesis in the human adrenal cortex.
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PMID:Dehydroepiandrosterone sulfate (DHEA-S) and 3', 5'-cyclic adenosine monophosphate (cAMP) production in a cultured human adrenocortical carcinoma cell line (SW-13). 284 Feb 74

Adrenocortical carcinoma tissue removed from a mildly hirsute 16-year-old girl was cultured in order to assess steroidogenesis and responsiveness of the cells to adrenocorticotropic hormone (ACTH), human chorionic gonadotropin (HCG), and insulin. The cells in culture produced large amounts of androstenedione and testosterone; however, production of cortisol, which was initially high, decreased with time. No aldosterone, estrone, or estradiol was produced in vitro. Both monolayer cells maintained for 6 weeks and organ culture explants maintained for over 3 days responded to ACTH (10(-7) M) with increased production of androgens (testosterone, androstenedione, dehydroepiandrosterone) but decreased production of cortisol as measured by radioimmunoassay of steroids in the culture media. Concomitant with decreased cortisol production was the enhanced formation of 11-deoxycortisol in cells exposed to ACTH, suggesting impaired 11 beta-hydroxylation. Tissue exposed to HCG (10(-7) M) in organ culture showed an increase in androgen production over control levels, but no significant effect of HCG on glucocorticoid production was found. Tumor cells differed in their androgen response to ACTH and HCG, with enhanced adrenal androgens in the presence of ACTH and more gonadal-type androgens after exposure to HCG. Insulin exposure had no effect on production of either androgen or glucocorticoid by tumor tissue in organ culture. Thus, this adrenocortical carcinoma showed marked androgen production in culture which was enhanced in different ways by ACTH and HCG. 11 beta-Hydroxylation was impaired with time in culture. No specific effect of insulin on steroidogenesis was noted.
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PMID:Effects of adrenocorticotropic hormone, human chorionic gonadotropin, and insulin on steroid production by human adrenocortical carcinoma cells in culture. 299 Jun 78

A spontaneously occurring rat adrenocortical carcinoma which produces corticosterone was maintained by transplantation. The carcinoma appeared to utilize corticosterone biosynthetic steps similar to those of the normal adrenal, but the tumor produced only about 1-10% as much corticosterone per unit tissue weight as nontumorous adrenal glands. The tumor demonstrated little or no increase in corticosterone production in response to adrenocorticotropic hormone (ACTH) either in vivo or in vitro. In normal adrenals, ACTH increases the activity of adenyl cyclase which catalyzes the conversion of adenosine triphosphate (ATP) to adenosine-3',5'-monophosphate (cyclic AMP), the latter then serving as an intracellular regulator of steroidogenesis. ACTH failed to increase cyclic AMP levels in the tumor in vivo or in slices in vitro, conditions under which there were 50- and 20-fold increases in nontumorous adrenals. However, in homogenates fortified with exogenous ATP, adenyl cyclase activity was comparable in the tumor and adrenals, and cyclic AMP formation was increased 3-fold by ACTH in each. As measured in homogenates, the tumor did not possess a greater ability to destroy cyclic AMP than did normal adrenals. Although ATP levels in the carcinoma were found to be considerably lower than those in normal adrenals, it was not clear that this finding can explain the inability of ACTH to increase cyclic AMP levels in intact tumor cells. While the failure to normally influence cyclic AMP levels in the carcinoma cells could be an important factor in the lack of a steroid response to ACTH, several lines of evidence suggest that the tumor possesses one or more additional abnormalities in the regulation of steroidogenesis. First, in the absence of ACTH stimulation, the tissue concentrations of cyclic AMP were comparable in the tumor and in nontumorous adrenals, but these cyclic AMP levels were associated with a lower level of steroidogenesis in the tumor. Second, tumor slices failed to increase corticosterone production when incubated with cyclic AMP, in contrast to 5-fold increases observed with nontumorous adrenals.
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PMID:Abnormal regulation of adenosine 3',5'-monophosphate and corticosterone formation in an adrenocortical carcinoma. 439 Apr 12

Three patients with primary aldosteronism due to adrenocortical carcinoma were studied, two with hyperaldosteronism alone and one also with hypercortisolism; in the later stages all three had hypersecretion of glucocorticoid and androgenic hormones. Although clinical presentations were similar to those of patients with benign adenoma, all had significantly higher concentrations of deoxycorticosterone and aldosterone and more profound hypokalemia. Stimulation with adrenocorticotropin in two patients showed a good cortisol response but no aldosterone response. The circadian rhythm for cortisol was normal but absent for aldosterone and deoxycorticosterone. Sequential 24-hour circadian studies in one patient showed that as the disease progressed, corticosterone and finally cortisol lost their circadian rhythms. Treatment with spironolactone, mitotane, or aminoglutethimide had transient clinical effects. The patients died 2 to 13 years later.
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PMID:Aldosterone-producing adrenocortical carcinoma. Preoperative recognition and course in three cases. 608 4

A case of a huge inoperable adrenocortical carcinoma which secreted testosterone without characteristic symptoms was treated with o,p'-DDD (2,2-bis(2-chlorophenyl-4 cholorophenyl) 1,1-dichloroethane). With this therapy, the tumor decreased in size which was confirmed by the computed tomography (CT). Eighteen months later, however, lethargy and logopathy appeared and the tumor grew again rapidly with the withdrawal of o,p'-DDD performed for the evaluation of these mental disturbances. The tumor then diminished gradually in size soon after the treatment was resumed and the above unfavorable symptoms were not developed again with the combined administration of a central nervous stimulant. During o,p'-DDD treatment, plasma testosterone and estrogen decreased, and plasma aldosterone also decreased but within the normal range. Plasma cortisol also tended to decrease despite hydrocortisone was administered. Plasma adrenocorticotropic hormone (ACTH) was maintained within the normal range for the first six months but then increased gradually. It decreased and became normal with the additional administration of hydrocortisone. The patient's normal menstruation at the preadministrative stage changed to oligomenorrhea, then amenorrhea after the treatment, but no endocrinological sign except for the menses was observed during the treatment.
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PMID:Adrenocortical carcinoma responded to treatment with o,p'-DDD--a case report. 609 39

Adenylate cyclase responses to pituitary hormones including adrenocorticotropic hormone (ACTH), biogenetic amines, prostaglandin E1 (PGE1), angiotensin II, and glucagon were evaluated in adrenocortical tumors and hyperplastic adrenal tissues, obtained from patients with Cushing's syndrome at surgery, and in normal adrenals. The adenylate cyclase of two normal adrenals was activated only by ACTH and PGE1 among the hormones tested, while that of two hyperplastic adrenal tissues due to excessive pituitary ACTH secretion was stimulated only by ACTH. Of five ACTH-responsive adrenocortical adenomas, in contrast, three were stimulated by norepinephrine, two by epinephrine, one by thyroid-stimulating hormone, and one by luteinizing hormone in addition to ACTH, indicating the presence of multiple receptors for hormones other than ACTH and PGE1 in these four tumors. The cyclase of an ACTH-unresponsive adrenocortical carcinoma ws activated only by PGE1 and not by other hormones including ACTH, whereas that of an ACTH-responsive adrenocortical nodular hyperplasia was stimulated by ACTH and glucagon but not by other hormones including PGE1. These results indicate the presence of multiple receptors for hormones other than ACTH and PGE1, the normal adrenocortical stimulants, in human adrenocortical tumors, particularly in adrenal adenomas, but not in normal and hyperplastic (of whichever an etiology) adrenocortical tissues, suggesting a functional alteration of the cellular membrane receptors in human adrenocortical tumors.
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PMID:Multiple hormone receptors in the adenylate cyclase of human adrenocortical tumors. 625 40

Immunoreactive plasma levels of the proopiolipomelanocortin-derived peptides, ACTH, beta-endorphin-lipotropin, and gamma 3MSH, were measured in patients with primary hyperaldosteronism, idiopathic hyperaldosteronism with bilateral adrenal hyperplasia, and dexamethasone-suppressible hyperaldosteronism. Plasma peptide concentrations in patient groups were not different from those in normal controls. Removal of aldosterone-producing adenomas in three patients and of an aldosterone-producing adrenocortical carcinoma in one patient did not affect plasma peptide concentrations. Furthermore, infusion of the opiate antagonist naloxone (0.2 mg/min) in one patient with bilateral adrenal hyperplasia had no effect on either plasma aldosterone or cortisol. These results suggest that the proopiolipomelanocortin-derived peptides are not overproduced in states of hyperaldosteronism.
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PMID:Plasma immunoreactive proopiolipomelanocortin-derived peptides in patients with primary hyperaldosteronism, idiopathic hyperaldosteronism with bilateral adrenal hyperplasia, and dexamethasone-suppressible hyperaldosteronism. 630 Jan 69


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