11.BETA.-hydroxylase in mitochondrial fractions of functioning and non-functioning adrenocortical tumors.

Abstract

NAGANUMA, H., OJIMA, M. and SASANO, N. 11/3-Hydroxylase in Mitochondrial Fractions of Functioning and Non-Functioning Adrenocortical Tumors. Tohoku J. exp. Med., 1988, 155 (1), 81-96The activity of 11/3-hydroxylase was investigated in the mitochondrial fractions of 19 surgically resected adrenocortical tumors associated with Cushing's syndrome (4 adenomas and 2 carcinomas), primary aldosteronism (8 adenomas), adrenogenital syndrome (AGS) (2 carcinomas) and no hormonal symptoms (3 adenomas). Five adrenal cortices from patients with mammary carcinoma, renal cell carcinoma and pheochromocytoma were used for the normal control. The activities of 11/3-hydroxylation of deoxycorticosterone and of 11-deoxycortisol in the control adrenal cortices were 0.66-2.16 pmole/mg protein/min (mean : 1.28 pmole/mg protein/min) and 0.25-0.77 pmole/mg protein/min (mean : 0.56 pmole/mg protein/min), respectively. The activities in adenomas and carcinomas associated with Cushing's syndrome were in the range of normal control. The activities in aldosteronomas were significantly higher in 4 cases than those of the normal control and in the range of the normal control in 4 cases, suggesting that the higher activity of 11/3-hydroxylase is one of the important factors causing mineralocorticoid excess. The activities in adrenocortical carcinomas with AGS were significantly lower, corroborating clinical findings of androgen excess with suppressed production of mineralocorticoid or glucocorticoid. The activities in two cases of nonfunctioning adenomas were in the range of the normal control, but the third case showed higher activity than the normal control. These results show that the abnormal activity of mitochondrial 11/3-hydroxylase exists in some aldosteronomas, non-functioning adrenocortical adenomas and carcinomas with AGS.

Steroid 21-hydroxylase and 17.ALPHA.-hydroxylase in microsomal fraction of functioning adrenocortical tumors.

“…11/8-hydroxylase activity was also in the range of normal controls in our previous report (Naganuma et al 1988). Other investigators documented the increased activity of various enzymes in Cushing's adenomas (Degenhart et al 1982;D'Agata et al 1987).…”

“…We have been studying the relationship between morphology and function of adrenocortical tumors to know whether the morphological abnormality cause following excessive production of steroid. We documented the abnormality of 11/3-hydroxylase and 18-hydroxylase in mitochondrial fraction of adrenocortical tumors previously (Naganuma et al 1988). In the present paper we report the abnormal activities of steroid 21-hydroxylase and 17a-hydroxylase in functioning adrenocortical tumors manifesting Cushing's syndrome, primary aldosteronism or AGS.…”

“…They mentioned the decreased activity of 17a-hydroxylase by the results of plasma steroids level, and Dufau et al (1968) have already reported it by enzyme assay in the tissue of aldosteronoma. Furthermore in our previous study 11, 3-hydroxylase and 18-hydroxylase activity in mitochondrial fractions of aldosteronomas were elevated (Naganuma et al 1988). These results support the excessive production of aldosterone (mineralocorticoids) in the patients with aldosteronoma.…”

Pure androgen-secreting adrenal tumor (PASAT): A rare case report of bilateral PASATs and a systematic review

“…In total, 48 studies met the inclusion criteria, including 40 case reports ( 8 – 47 ) and 8 articles ( 48 – 55 ). Forty case reports including 41 adult PASAT cases are summarized in Table 2 , and 8 articles containing adult PASAT patients are summarized in Table 3 .…”