Pregnanetriolone, or 11-ketopregnanetriol, is a steroid hormone.
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| IUPAC name
3R,5R,8S,9S,10S,13S,14S,17R)-3,17-dihydroxy-17-[(1S)-1-hydroxyethyl]-10,13-dimethyl-2,3,4,5,6,7,8,9,12,14,15,16-dodecahydro-1H-cyclopenta[a]phenanthren-11-one
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| Other names
5β-Pregnane-3α,17α,20α-triol, 11-Ketopregnanetriol, 5beta-Pregnane-3alpha,17alpha,20alpha-triol-11-one
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| Identifiers | |
3D model (JSmol)
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| ChEBI | |
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| KEGG | |
PubChem CID
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| Properties | |
| C21H34O4 | |
| Molar mass | 350.499 g·mol−1 |
Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa).
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Clinical significance edit
There is no or little urinare excretion of pregnanetriolone in the urine in healthy people.[1]
Pregnanetriolone is a metabolite of 21-deoxycortisol.[1][2]
In patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH), the daily excretion exceeds 100 μg. ACTH stimulation increased excrement even further. It has been concluded since at least 1974 that the excretion of pregnenolone in urine after ACTH stimulation test can help detect heterozygous carriers of congenital adrenal hyperplasia caused by 21-hydroxylase deficiency (non-classical forms of CAH).[3][4][5][6] These conclusions were confirmed by later studies.[1][7]
Pregnanetriolone can be used as a marker screening and for monitoring of treatment in infants with classical forms of CAH.[8]
See also edit
References edit
- ^ a b c Homma K, Hasegawa T, Takeshita E, Watanabe K, Anzo M, Toyoura T, Jinno K, Ohashi T, Hamajima T, Takahashi Y, Takahashi T, Matsuo N (December 2004). "Elevated urine pregnanetriolone definitively establishes the diagnosis of classical 21-hydroxylase deficiency in term and preterm neonates". J Clin Endocrinol Metab. 89 (12): 6087–91. doi:10.1210/jc.2004-0473. PMID 15579762.
- ^ Greaves RF, Kumar M, Mawad N, Francescon A, Le C, O'Connell M, Chi J, Pitt J (October 2023). "Best Practice for Identification of Classical 21-Hydroxylase Deficiency Should Include 21 Deoxycortisol Analysis with Appropriate Isomeric Steroid Separation". Int J Neonatal Screen. 9 (4): 58. doi:10.3390/ijns9040058. PMC 10594498. PMID 37873849.
- ^ Homoki J, Teller WM, Fazekas AT (April 1976). "A test for heterozygocity of 21-hydroxylase deficiency: preliminary report". Human Genetics. 32 (1): 35–41. doi:10.1007/BF00569974. PMID 177352. S2CID 20559786.
- ^ Halperin G, Finkelstein M (March 1967). "Biosynthesis of pregnanetriolone and pregnanetetrol in congenital adrenal hyperplasia". Acta Endocrinologica. 54 (3): 439–51. doi:10.1530/acta.0.0540439. PMID 6071362.
- ^ Thomas FJ, Steinbeck AW (April 1969). "Quantitative estimation of urinary pregnanetriol, pregnanetriolone, tetrahydro S and delta-5-pregnenetriol in the investigation of adrenocortical function". Acta Endocrinologica. 60 (4): 657–68. doi:10.1530/acta.0.0600657. PMID 5819067.
- ^ Zachmann M, Prader A (March 1978). "Unusual heterozygotes of congenital adrenal hyperplasia due to 21-hydroxylase deficiency". Acta Endocrinologica. 87 (3): 557–65. doi:10.1530/acta.0.0870557. PMID 580145.
- ^ Alonso-Fernández JR (September 2016). "Pregnanetriolone in paper-borne urine for neonatal screening for 21-hydroxylase deficiency: The place of urine in neonatal screening". Molecular Genetics and Metabolism Reports. 8: 99–102. doi:10.1016/j.ymgmr.2016.08.006. PMC 4992010. PMID 27570738.
- ^ Kamrath C, Friedrich C, Hartmann MF, Wudy SA (July 2023). "Metabotypes of congenital adrenal hyperplasia in infants determined by gas chromatography-mass spectrometry in spot urine". J Steroid Biochem Mol Biol. 231: 106304. doi:10.1016/j.jsbmb.2023.106304. PMID 36990162. S2CID 257775623.
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