• Animal cells and systems
• /
• v.9 no.1
• /
• pp.19-25
• /
• 2005

This study was conducted to determine the effect of dietary salt on the synthesis of glucocorticoids in the adrenal cortex of mice. Mice had ad libitum access to 3% sodium chloride as the only drinking fluid (high salt diet) for either 4 days or 4 weeks. Adrenocortical expression of cytochrome P450 11beta-hydroxylase, a major regulatory enzyme in the biosynthesis of glucocorticoids, was examined by immunohistochemistry and western blot analysis. Ultrastructure of adrenocortical cell and plasma level of corticosterone were analyzed as well. Size and density of lipid droplets in the cortical cell were increased by high salt diet. Four days of high salt diet decreased P450 11beta-hydroxylase in the adrenal cortex, but 4 weeks increased it. Plasma level of corticosterone changed in parallel with the Cortical level of P450 11 beta-hydroxylase. These results suggest that high salt diet may modulate the biosynthesis of glucocorticoids, at least partly, via regulating the expression of P450 11beta-hydroxylase in adrenocortical cells.

• Korean Journal of Microbiology
• /
• v.49 no.1
• /
• pp.95-98
• /
• 2013

Following construction of expression vectors in Escherichia coli, a new procedure involving two-step column purifications with a Fast Performance Liquid Chromatography System was developed for purification of the oxygenase component of alkylphenol hydroxylase of Pseudomonas alkylphenolia. From 50 g wet cake of recombinant E. coli BL21(DE3)(pJJPMO2) cells, 110 mg of pure protein in a heterodimeric form containing a stoichiometric amount of iron were obtained and it exhibited a specific activity of 147 nmole/min/mg.

• Clinical and Experimental Pediatrics
• /
• v.51 no.9
• /
• pp.1018-1022
• /
• 2008

Testicular adrenal rest tumors are a well-known complication in male patients with congenital adrenal hyperplasia. Corticosteroid suppressive therapy usually results in the regression of these tumors. We describe a patient with 21-hydroxylase deficiency who developed bilateral testicular masses. Despite steroid suppressive therapy, the tumors did not regress and hormonal control was poor. Consequently, bilateral partial orchiectomies were performed.

• Journal of Plant Biology
• /
• v.35 no.3
• /
• pp.191-195
• /
• 1992

Changes in activity of gibberellin C20-hydroxylase which converts $[^{14}C]GA_{12}\;to\;GA_{l5}$ were studied during seed maturation using the partially purified enzyme preparations of two cultivars, Kentucky Wonder (normal) and Masterpiece (dwarf) of Phaseolus vulgaris. The preparations obtained by methanol precipitation and hydrophobic interaction chromatography efficiently converted; $GA_{l2}\;to\;GA_{4},\;via\;GA_{15},\;GA_{24}\;and\;GA_{9};\;GA_{20}\;to\;GA_{1},\;GA_{5}\;and\;GA_{6}$. The activities of C20-hydroxylase which converts $GA_{l2}\;to\;GA_{l5}$ were almost the same in both cultivars. The C20-hydroxylase activity per protein reached a maximum at the very early immature seed stage, followed by a subsequent rapid decrease during seed maturation, whereas the enzyme specific activity per seed reached a maximum at 21 days after flowering, and showed a similar fiuctuation to that of the 313-hydroxylase which converts $GA_{20}\;to\;GA_{l}$ during seed maturation.ration.

• Clinical and Experimental Reproductive Medicine
• /
• v.42 no.2
• /
• pp.72-76
• /
• 2015

$17{\alpha}$-hydroxylase and 17,20-lyase are enzymes encoded by the CYP17A1 gene and are required for the synthesis of sex steroids and cortisol. In $17{\alpha}$-hydroxylase deficiency, there are low blood levels of estrogens, androgens, and cortisol, and resultant compensatory increases in adrenocorticotrophic hormone that stimulate the production of 11-deoxycorticosterone and corticosterone. In turn, the excessive levels of mineralocorticoids lead to volume expansion and hypertension. Females with $17{\alpha}$-hydroxylase deficiency are characterized by primary amenorrhea and delayed puberty, with accompanying hypertension. Affected males usually have female external genitalia, a blind vagina, and intra-abdominal testes. The treatment of this disorder is centered on glucocorticoid and sex steroid replacement. In patients with $17{\alpha}$-hydroxylase deficiency who are being raised as females, estrogen should be supplemented, while genetically female patients with a uterus should also receive progesterone supplementation. Here, we report a case of a 21-year-old female with $17{\alpha}$-hydroxylase deficiency who had received inadequate treatment for a prolonged period of time. We also include a brief review of the recent literature on this disorder.

• Journal of Plant Biology
• /
• v.35 no.3
• /
• pp.185-190
• /
• 1992

Changes in activity of gibberellin $3{\beta}-hydroxylase$ which converts $[^3H]GA_{20}\;to\;GA_1$ were studied during seed maturation using partially purified enzyme preparations of two cultivars, Kentucky Wonder (normal) and Masterpiece (dwarf) of Phaseolus vulgaris. The specific activity of $3{\beta}-hydroxylase$ per seed reached maximum at 21 days after flowering and subsequently decreased during seed maturation in both cultivars. The ratios of conversion of $[17-^{13}C,\;^3H_2]GA_{20}\;to\;GA_1.\;GA_5,\;and\;GA_6$ by the same amount of $3{\beta}-hydroxylase$ were almost identical. Epoxidation of $GA_5\;to\;GA_6$ is also catalyzed by the partially purified $3{\beta}-hydroxylase$ preparation(Kobayashi et aI., 1991) and the conversion was inhibited by the substrates of $3{\beta}-hydroxylase$. These results suggest that the same enzyme might catalyze $3{\beta}-hydroxylase{\;}of{\;}GA_{20}\;to\;GA_1$ and epoxidation of $GA_5\;to\;GA_6$..

• Preventive Nutrition and Food Science
• /
• v.13 no.2
• /
• pp.61-65
• /
• 2008

To evaluate the effect of ground cherry extract on the activity of aniline hydroxylase, we gave ground cherry extract in doses of 100, 200 or 400 mg/kg i.p to mice for 1, 2 or 4 days. The aniline hydroxylase activity in the group treated with ground cherry extract increased in a dose dependant manner in all experimental groups compared with the control group, and was significantly higher in the group treated with ground cherry extract at a dose of 200 mg/kg, which also exhibited a time dependant increase over 4 days. Enzyzme kinetic analysis was performed for hepatic aniline hydroxylase activity in the group treated with 200 mg/kg for 4 days. There was no change of the Km values for aniline hydroxylase between the experimental group and the control group, but the Vmax values for aniline hydroxylase was 21% lower in the experimental group compared with the control. The experimental group also showed lower lipid peroxide and reduced glutathione content, and there were no significant difference in serum alanine aminotransferase activity between the experimental group and the control. Aniline was injected into both the experimental group mice treated with ground cherry extract at a dose of 200 mg/kg for 4 days and the control group, and then the level of blood aniline was assayed at 1hr. The level of blood aniline was lower in the experimental than the control group. This study suggests that ground cherry extract induces hepatic aniline hydroxylase activity and might accelerate the scavenging system of reactive oxygen species. It is likely that ground cherry extract influences the metabolism of xenobiotics by activating AH activity substituted for CYP2E1.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.16 no.2
• /
• pp.70-78
• /
• 2016

21-hydroxylase deficiency (21-OHD), most common form of congenial adrenal hyperplasia, is categorized into classical forms, including the salt-wasting (SW) and the simple virilizing (SV) types, and nonclassical (NC) forms based on the severity of the disease. Newborn screening for 21-OHD has been performed in Korea since 2006. $17{\alpha}$-hydroxyprogesterone (17-OHP) is a marker for 21-OHD and is measured using a radioimmunoassay or a fluoroimmunoassay. Premature and low birth weight infants are likely to give false positive 17-OHP findings, therefore, cutoff values for these infants should be determined based on gestational weeks or birth weight. ACTH simulation test is helpful when the 17-OHP shows equivocal increase, and it is gold standard for diagnosis of NC type. Recently, liquid chromatography linked with tandem mass spectrometry was developed for rapid, highly specific, and sensitive analysis of multiple analytes. Molecular analysis of CYP21A2 is useful for confirming diagnosis of mild SV or NC type, predicting prognoses, and genetic counseling. In order to make newborn screening for 21-OHD more efficient, early detection of boy with SW type, early determination of girl with ambiguous genitalia, detection of NC type, and overcoming of false positive in premature and low birth weight infants should be considered. Above all, early treatment should be started when the patient is suspected as having 21- OHD clinically before confirming the diagnosis to prevent adrenal crisis. Here, author reviewed recent articles of guideline and proposed guideline for 21-OHD.

• Korean Journal of Microbiology
• /
• v.27 no.4
• /
• pp.366-372
• /
• 1989

Twenty-one strains were tested for 11$\beta$-hydroxylation of Reichstein's substance S. Four fungi exhibited ability for the reaction, among which Pellicularia fillamentosa showed the highest activity. The 11$\beta$-hydroxylase of this fungus was proved to be induced by the substrate, cycloheximide reducing significantly the activity of the enzyme. Range of optimum pH for the 11$\beta$-hydroxylation was broad and found to be 2.0-8.0. Test of the enzyme activity at different growing stages, from spore to mycelia, showed that the branching stage of hyphae and the mature mycelial stage were the most effective for the Reichstein's substance S transformation. However, 11$\beta$-hydroxylase in the intact spore was turned out to be uninducible with the substrate.

• Clinical and Experimental Pediatrics
• /
• v.60 no.2
• /
• pp.31-37
• /
• 2017

Steroid 21-hydroxylase deficiency is the most prevalent form of congenital adrenal hyperplasia (CAH), accounting for approximately 95% of cases. With the advent of newborn screening and hormone replacement therapy, most children with CAH survive into adulthood. Adolescents and adults with CAH experience a number of complications, including short stature, obesity, infertility, tumor, osteoporosis, and reduced quality of life. Transition from pediatric to adult care and management of long-term complications are challenging for both patients and health-care providers. Psychosocial issues frequently affect adherence to glucocorticoid treatment. Therefore, the safe transition of adolescents to adult care requires regular follow-up of patients by a multidisciplinary team including pediatric and adult endocrinologists. The major goals for management of adults with 21-hydroxylase deficiency are to minimize the long-term complications of glucocorticoid therapy, reduce hyperandrogenism, prevent adrenal or testicular adrenal rest tumors, maintain fertility, and improve quality of life. Optimized medical or surgical treatment strategies should be developed through coordinated care, both during transition periods and throughout patients' lifetimes. This review will summarize current knowledge on the management of adults with CAH, and suggested appropriate approaches to the transition from pediatric to adult care.