In individuals with GRA, there is unequal crossing over so that the 5 regulatory region of the 11-hydroxylase gene is fused to the coding region of the aldosterone synthase.The product of this hybrid gene is aldosterone synthase that is ACTH-sensitive in the zona fasciculata of the adrenal gland.Although in normal subjects, ACTH accelerates the first step of aldosterone synthesis, ACTH normally has no effect on the activity of aldosterone synthase. However, in subjects with glucocorticoid-remediable aldosteronism, ACTH increases the activity of existing aldosterone synthase, resulting in an abnormally high rate of aldosterone synthesis and hyperaldosteronism. Diagnosis
Genetic testing is done to ascertain that the individual in question does indeed have the condition

Treatment
In GRA, the hypersecretion of aldosterone and the accompanying hypertension are remedied when ACTH secretion is suppressed by administering glucocorticoids.Dexamethasone, spironolactone and eplerenone have been used in treatment. See also
Inborn errors of steroid metabolism
Hyperaldosteronism
Pseudohyperaldosteronism
Apparent mineralocorticoid excess syndrome
Aldosterone and aldosterone synthase

References


== External links ==
Necitumumab (INN) is a recombinant human IgG1 monoclonal antibody used as an antineoplastic, which is manufactured by Eli Lilly. It binds to the epidermal growth factor receptor (EGFR). The US FDA approved necitumumab under the brand name Portrazza for use with gemcitabine  and cisplatin  in previously untreated metastatic squamous non-small-cell lung carcinoma (NSCLC). It was counterproductive in non-squamous non-small-cell lung carcinoma. == References ==