• Clinical and Experimental Reproductive Medicine
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• 제42권2호
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• pp.72-76
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• 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.

• Archives of Pharmacal Research
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• 제20권6호
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• pp.525-528
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• 1997

Steroid $9{\alpha}$.-hydroxylase is a key enzyme system in steroid nucleus degradation in company with ${\Delta}$-dehydrogenase. To examine $9{\alpha}$-hydroxylase activity during microbial transformation of steroids, 9(11)-dehydro-$17{\alpha}$-methyl-testosterone was adopted as a stable substrate for preventing the rupture of steroid nucleus. Using Nocardia restrictus ATCC 14887 capable of introducing a $9{\alpha}$-hydroxyl group into steroids, $9{\alpha}$,$11{\alpha}$-oxido-$17{\beta}$-hydroxy-$17{\alpha}$-methyl-4-androstene-3-one and $9{\alpha}$-hydroxyl group into steroids,$9{\alpha}$,$11{\alpha}$-oxido-$17{\beta}$-hydroxy-$17{\alpha}$-methyl-1,4-androstadiene-3- one were obtained. These microbiologically transformed products could be used as reference compounds in the enzyme assay.

• 약학회지
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• 제41권5호
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• pp.638-646
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• 1997

The steroid 9${alpha}$-hydroxylase activity has been detected in cytosol fraction, $100,00{\times}g$ supernatant of cell free extract of Mycobacterium fortuitum. The activity was not linear with protein concentration in the assay suggesting 9${alpha}$-hydroxylase is a multicomponent enzyme. The 9${alpha}$-hydroxylase system was partially purified through fractional saturation of ammonium sulfate, strong anion exchange (Mono Q) column chromatography, gel filtration (Superose 12) column chromatography, and testosterone affinity gel chromatography. Ammonium sulfate 50~60% saturated fraction of the cytosol gave 9${alpha}$-hydroxylase activity. For further purification, the half-saturated ammonium sulfate fraction was applied to Mono Q, Superose 12, or affinity gel column. The purification factors of 9${alpha}$-hydroxylase containing fraction after Mono Q, Superose 12, and affinity gel chromatography was 13, 11, and 17 respectively.

• 한국발생생물학회지:발생과생식
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• 제10권2호
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• pp.127-133
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• 2006

스테로이드 합성효소 중 $17\;{\alpha}-hydroxylase/17,20-lyase(P450_{C17})$는 progesterone을 $17\;{\alpha}-hydroxyprogesterone$을 거쳐 androstenedione으로 변환을 담당하는 효소이다. 양서류 난소에서 스테로이드 합성의 분자적 조절과정의 연구에 사용할 목적으로 북방산 개구리(Rana dybowskii) 난소에서 $P450_{C17}$ cDNA를 클로닝 하였다. 북방산 개구리 난포세포의 cDNA library 검색을 통해 분리된 약 2.5kb의 cDNA는 529개의 아미노산을 가진 단일 번역틀을 가지고 있었다. 개구리 $P450_{C17}$의 아미노산 서열은 Xenopus와는 76%, 닭과는 63%, 그리고 사람과는 약 45%의 동일성을 보여 주였고, 동시에 진화적으로 척추동물에서 매우 잘 보존된 아미노산 서열을 가지고 있었다. 노던 분석에서 개구리의 $P450_{C17}$ 전사체는 난소에서만 2.5kb와 3.6kb 크기의 두 종류가 발견되었다. 그리고 개구리 Rana $P450_{C17}$ cDNA는 비스테로이드 합성 세포인 COS-1세포에서 분명한 $17\;{\alpha}-hydroxylase/17,20-lyase$ 활성을 주었다. 따라서 클로닝된 개구리 $P450_{C17}$ 유전자는 양서류의 난소에서 스테로이드 합성의 분자적 기작을 연구하는데 매우 유용할 것으로 사료된다.

• Clinical and Experimental Reproductive Medicine
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• 제33권2호
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• pp.133-138
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• 2006

46,XY 핵형인 남성에게서 $17{\alpha}$-hydroxylase의 결핍이 있을 경우 성호르몬의 생성장애로 인해 남성가성반음양에 의한 여성 표현형을 보이게 된다. 코티졸이 결핍되면 부신피질자극호르몬의 증가 및 이로 인한 광불무신피질호르몬의 합성이 증가되어 저레닌성 고혈압, 저칼륨혈증이 발생한다. 41세 된 여자 환자가 원발성 무월경과 고혈압을 주소로 내원하였다. 46,XY 핵형을 보였으며, 호르몬 검사상, 혈중 에스트라디올, 테스토스테론, 레닌, 코티졸이 감소되어 있으며 부신피질자극호르몬 및 deoxycorticosterone이 증가되어 있어 $17{\alpha}$-hydroxylase 결핍에 의한 남성가성반음양으로 진단되었다. 이 질환의 치료로 복강경을 이용한 양측 고환적출술을 시행하였고, 부신피질호르몬과 항고혈압 재제, 에스트로젠을 투여하였다. 본 연구에서는 위 증례에 대하여 간단한 문헌고찰과 함께 보고하는 바이다.

• 한국응용약물학회:학술대회논문집
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• 한국응용약물학회 2007년도 Proceedings of The Convention
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• pp.57-64
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• 2007

Metabolic interactions of the three major cannabinoids, ${\Delta}^9$-tetrahydrocannabinol (THC), cannabidiol (CBD), and cannabinol (CBN) with steroid hormones were investigated. These cannabioids concentration-dependently inhibited $3{\beta}$-hydroxysteroid dehydrogenase and $17{\alpha}$-hydroxylase in rat adrenal and testis microsomes. CBD and CBN were the most potent inhibitors of $3{\beta}$-phydroxysteroid dehydrogenase and progesterone $17{\alpha}$-hydroxylase, respectively, in rat testis microsomes. Three cannabinoids highly attenuated hCG-stimulated testosterone production in rat testicular interstitial cells. These cannabinoids also decreased in levels of mRNA and protein of StAR in the rat testis cells. These results indicate that the cannabinoids could interact with steroid hormones, and exert their modulatory effects on endocrine and testicular functions. Metabolic interaction of a THC metabolite, $7{\beta}$-hydroxy-${\Delta}^8$-THC with steroids is also investigated. Monkey liver microsomes catalyzed the stereoselective oxidation of $7{\beta}$-hydroxy-${\Delta}^8$-THC to 7-oxo-${\Delta}^8$-THC, so-called microsomal alcohol oxygenase (MALCO). The reaction is catalyzed by CYP3A8 in the monkey liver microsomes, and required NADH as well as NADPH as an efficient cofactor, and its activity is stimulated by some steroids such as testosterone and progesterone. Kinetic analyses revealed that MALCO-catalyze reaction showed positive cooperativity. In order to explain the metabolic interaction between the cannabinoid metabolite and testosterone, we propose a novel kinetic model involving at least three binding sites for mechanism of the metabolic interactions.

• 한국미생물생명공학회:학술대회논문집
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• 한국미생물생명공학회 1979년도 춘계학술대회
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• pp.116-116
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• 1979

In the experiment of cholesterols and steroidal compounds. gas chromatography has been widely used to determine the compounds. Without the facility, we could determine the amount of 17-ketosteroids in the use of t. 1. c technique. In the muicrobial conversion of cholesterol to 17-ketsoteroids, $\alpha,$ $\alpha'-dipyridyl$ which might be a inhibitor of $9\alpha-hydroxylase$ of steroid skeleton was added to microbial culture broth. The inhibitor contaminated due to its solubility in organic solvents and hindered the determination of 17-ketost eroids on t.1. c in all the process of the experiment. we successfully determined the 17-ketosteroids by the use of Ag$^{+}$ band on t. 1. c. plate.e.

• 한국동물학회:학술대회논문집
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• 한국동물학회 1996년도 한국생물과학협회 국제학술대회
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• pp.145.1-145
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• 1996

No Abstract, See Full Text

• 한국동물학회:학술대회논문집
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• 한국동물학회 1997년도 한국생물과학협회 학술발표대회
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• pp.191.1-191
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• 1997

No Abstract, See Full Text

• Asian-Australasian Journal of Animal Sciences
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• 제20권12호
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• pp.1832-1842
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• 2007

Cytochrome P450 aromatase is responsible for the biosynthesis of estrogen. It is also responsible for the endogenous production of 19-nortestosterone (nandrolone), an anabolic androgen unique to pigs. Plasma concentrations of 19-nortestosterone are highest between two and four weeks after birth in male pigs. In the present study, the physiology of 19-nortestosterone was investigated by measuring the mRNA levels of steroidogenic enzymes, estrogen receptors and androgen receptor in the tissues of growing pigs. The expression of aromatase, 17${\alpha}$-hydroxylase and 3${\beta}$-hydroxysteroid dehydrogenase in the testes of male piglets increased between birth and two weeks of age, and then decreased progressively. Similar developmental expressional patterns were observed for 17${\alpha}$-hydroxylase and 3${\beta}$-hydroxysteroid dehydrogenase in the ovaries of female piglets, but without significant aromatase expression. The major form of aromatase expressed in the testes of piglets was identified as type I. Expression of estrogen receptor-${\alpha}$ and -${\beta}$and androgen receptor genes was also detected in both testes and ovaries. A transient elevation of androgen receptor mRNA in male piglets at two weeks of age was also observed in testes. Significant expression of the androgen receptor gene, but not of estrogen receptor-${\alpha}$ and -${\beta}$ genes, was also demonstrated in adipose tissue and muscle. We conclude that the observed increase in the testicular expression of aromatase in male pigs could account for the production of large amounts of 19-nortestosterone at between two and four weeks of age in males. Androgen receptor and 19-nortestosterone appeared to be important for testicular development and might contribute to sexual dimorphism in body composition and muscle development in juvenile pigs.