The human adrenal cortex: growth control and disorders

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Term Occurence Count Dictionary
Li-Fraumeni syndrome 2 endocrinologydiseases
adrenal insufficiency 2 endocrinologydiseases
cortisol 23 endocrinologydiseasesdrugs
multiple endocrine neoplasia 1 endocrinologydiseases
testosterone 1 endocrinologydiseasesdrugs
mitotane 2 endocrinologydiseasesdrugs
Insulin 1 endocrinologydiseasesdrugs
arginine vasopressin 1 endocrinologydiseasesdrugs
congenital adrenal hyperplasia 1 endocrinologydiseases
diabetes mellitus 1 endocrinologydiseases
hypoglycemia 1 endocrinologydiseases
hypokalemia 1 endocrinologydiseases

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Select Drug Character Offset Drug Term Instance
Insulin 36645 [126].There are well-established molecular markers of great clinical importance for adrenocortical tumors. Insulin -like growth factor (IGF2) expression is associated with a malignant ACC phenotype [139]. In fact, it
arginine vasopressin 17017 241-amino acid precursor, POMC. ACTH is secreted by the anterior pituitary under the influence of CRH, arginine vasopressin (AVP) and some cytokines [50]. CRH secretion from the hypothalamus is regulated by circadian rhythms
cortisol 3258 accumulation in the cytoplasm, resembling that of active steroidogenic cells with the capacity for cortisol production. At approximately the 30th week of gestation, the fetal adrenal gland resembles a rudimentary
cortisol 9133 increased the effect of ACTH stimulation on the levels of dehydroepiandrosterone sulfate (DHEA-S) and cortisol in the culture medium. Moreover, the presence of fibronectin in the substrate enhanced the secretion
cortisol 9274 the presence of fibronectin in the substrate enhanced the secretion of DHEA-S but impaired that of cortisol [26]. In the DZ, laminin protected cells from apoptosis induced by angiotensin 2 (Ang II), which is
cortisol 14614 adrenal gland is steroid hormone production. Three main types of hormones are produced: glucocorticoids ( cortisol , corticosterone), mineralocorticoids (aldosterone, deoxycorticosterone) and androgens (sex steroids).
cortisol 16469 and salivary glands.GLUCOCORTICOID SECRETION: THE HYPOTHALAMUS-PITUITARY-ADRENAL (HPA) AXISIn humans, cortisol is the principal glucocorticoid produced by the human ZF under the control of ACTH, increasing blood
cortisol 25549 stimulus.ADRENOCORTICAL HYPERPLASIACongenital adrenal hyperplasia (CAH)CAH is an inherited enzymatic deficiency in cortisol synthesis. The deficiency in cortisol production results in excessive secretion of ACTH, which in turn
cortisol 25587 hyperplasia (CAH)CAH is an inherited enzymatic deficiency in cortisol synthesis. The deficiency in cortisol production results in excessive secretion of ACTH, which in turn cannot block cortisol synthesis, leading
cortisol 25674 deficiency in cortisol production results in excessive secretion of ACTH, which in turn cannot block cortisol synthesis, leading to enlargement of the adrenal gland. The more severe forms (i.e., complete enzymatic
cortisol 26787 deficiency. In nonclassic CYP21A2 deficiency, the alleles preserve some enzyme activity as well as cortisol and aldosterone production [88].The other form of CAH is 11β-hydroxylase (CYP11B1) deficiency, which
cortisol 27003 represents approximately 8% of all CAH cases [89]. Patients with this form of CAH also exhibit decreased cortisol synthesis and adrenal androgen overproduction. In contrast to CYP21A2, patients with CYP11B1 deficiency
cortisol 28314 deficiency [94].Deficiency in 17α-hydroxylase (CYP17A1) is rare and results in no production of either cortisol or androgens, but the progesterone and aldosterone pathways are not affected. CYP17A1 is encoded by
cortisol 30160 their ligands mimics the result of ACTH binding to MC2R, activating the PKA pathway and increasing cortisol production [101]. Additionally, the presence of ectopic POMC and ACTH in a subpopulation of cells within
cortisol 30612 PKA pathway and may act in autocrine and paracrine manners [103], allowing a certain independence of cortisol synthesis in PMAH cells.The molecular causes of PMAH have not been completely established, but several
cortisol 30820 several studies have tried to find a common event leading to the formation of macronodules and variable cortisol secretion. For example, mutations in the GNAS and MC2R genes have been reported, but they are present
cortisol 32403 syndrome (CS), which originates from chronic exposure to variable amounts of glucocorticoids, mainly cortisol [112].ADRENOCORTICAL ADENOMAS (ACA)Cortisol-producing adenomas are characterized by abnormally high
cortisol 32599 abnormally high levels of cAMP/protein kinase A (PKA) pathway activation. PKA is the main regulator of cortisol production and proliferation in adrenocortical cells [113]. PKA is composed of four subunits, two catalytic
cortisol 33103 factors in the nucleus, culminating in the transcription of steroidogenic enzymes and the synthesis of cortisol [114]. Mutations in the gene encoding the alpha catalytic subunit of protein kinase A (PRKACA) have
cortisol 33527 These mutations constitutively activate the cAMP pathway, resulting in abnormal PKA activity, increased cortisol production, and tumor development. According to Lacroix and coworkers [118], the absence of mutations
cortisol 33673 According to Lacroix and coworkers [118], the absence of mutations in the PRKACA gene in cases of low- cortisol -producing adenomas may explain why they rarely lead to CS over time [112].Adrenocortical adenomas have
cortisol 34516 involved in the formation of these tumors through the cAMP/PKA signaling pathway, stimulating both cortisol secretion and cell proliferation.Similar to adrenocortical adenomas, aldosterone-producing adenomas
cortisol 35982 architecture, and vascular, sinusoid and capsule invasion [130],[131]. ACC can produce aldosterone, cortisol , androgens or estrogens, as described in Table 1[132],[133]. Alterations in the steroidogenic profile
cortisol 47027 (HSD3B2) [201], a steroidogenic enzyme expressed in the ZG and ZR, which is involved in aldosterone and cortisol synthesis. This finding reinforces the hypothesis of disturbances in fetal differentiation. Another
cortisol 49861 factors (TFs) in the nucleus that will increase the transcription of steroidogenic enzymes, leading to cortisol production. In addition to this classic signaling, PMAH cells can be abnormally regulated by aberrant
mitotane 36391 adrenocortical mass [137],[138]. An adjuvant treatment for patients with ACC is the administration of mitotane (dichlorodiphenyldichloroethane, o,p'-DDD), whose accepted levels range from 14-20 mg/dl and whose common
mitotane 37439 pathway has been proposed as a promising treatment [142], and in combination with the administration of mitotane , it reduces tumor growth [143]. Moreover, the methylation levels of the IGF2 promoter region may be
testosterone 17674 [51]. These androgens have weak activity, but they are precursors for peripheral tissue conversion to testosterone and estrogens such as estradiol [52]. These androgens and precursor steroids produced in the human adrenal
Select Disease Character Offset Disease Term Instance
Li-Fraumeni syndrome 35533 and are more frequent in women [124]-[126]. ACCs may be associated with hereditary disorders such as Li-Fraumeni syndrome , multiple endocrine neoplasia type 1 (MEN1) and Lynch syndrome, which has a prevalence between 1-7%
Li-Fraumeni syndrome 44488 adrenocortical tumors are more frequently associated with Beckwith-Wiedemann syndrome [187],[188] and Li-Fraumeni syndrome (LFS) [189],[190]. Beckwith-Wiedemann syndrome causes adrenocortical hyperplasia and various neoplasms
adrenal insufficiency 5027 unclear. The deletion of exon 2 of Dax1 resulted in animals with normal adrenal function [12]; however, adrenal insufficiency develops with time in aging Dax1-deleted mice [13]. Dax1 is expressed in a population of stem cells
adrenal insufficiency 26270 deficiencies as well as androgen excess (virilization). Nonclassic CYP21A2 deficiency does not produce adrenal insufficiency but is associated with premature puberty, hirsutism, acne, and irregular menses due to excess androgen.
congenital adrenal hyperplasia 28726 mutations are present due to founder effects [96].The most severe defect in steroidogenesis is lipoid congenital adrenal hyperplasia (LCAH), in which the patients' adrenal glands are greatly enlarged and replete with lipids. This type
diabetes mellitus 50689 ACCs.SecretionIncidenceSymptomsReferencesAldosteronerareHypertension, heart diseaseVilela and Almeida ([132]), Akerstrom et al. ([133])Cortisol50%-80%Osteoporosis, diabetes mellitus , muscle weakness, plethoraElse et al. ([129])Androgen40%-60%Increased libido, acne, male baldness, virilization,
hypoglycemia 17192 hypothalamus is regulated by circadian rhythms and by stressors acting on the hypothalamus, such as hypoglycemia , hypotension and fever. The secretion of both CRH and ACTH is inhibited by glucocorticoids in negative
hypokalemia 27156 overproduction. In contrast to CYP21A2, patients with CYP11B1 deficiency present with hypertension and sometimes hypokalemia . The CYP11B1 gene is located on chromosome 8q21-22, approximately 40 kb from the homologous CYP11B2,
multiple endocrine neoplasia 35555 in women [124]-[126]. ACCs may be associated with hereditary disorders such as Li-Fraumeni syndrome, multiple endocrine neoplasia type 1 (MEN1) and Lynch syndrome, which has a prevalence between 1-7% in patients with ACCs [127]-[129].

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