Chemistry:Pregnanetriolone
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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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3D model (JSmol)
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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). | |
| Infobox references | |
Pregnanetriolone, or 11-ketopregnanetriol, is a steroid hormone.
Clinical significance
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
References
- ↑ 1.0 1.1 1.2 "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. December 2004. doi:10.1210/jc.2004-0473. PMID 15579762.
- ↑ "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. October 2023. doi:10.3390/ijns9040058. PMID 37873849.
- ↑ "A test for heterozygocity of 21-hydroxylase deficiency: preliminary report". Human Genetics 32 (1): 35–41. April 1976. doi:10.1007/BF00569974. PMID 177352.
- ↑ "Biosynthesis of pregnanetriolone and pregnanetetrol in congenital adrenal hyperplasia". Acta Endocrinologica 54 (3): 439–51. March 1967. doi:10.1530/acta.0.0540439. PMID 6071362.
- ↑ "Quantitative estimation of urinary pregnanetriol, pregnanetriolone, tetrahydro S and delta-5-pregnenetriol in the investigation of adrenocortical function". Acta Endocrinologica 60 (4): 657–68. April 1969. doi:10.1530/acta.0.0600657. PMID 5819067.
- ↑ "Unusual heterozygotes of congenital adrenal hyperplasia due to 21-hydroxylase deficiency". Acta Endocrinologica 87 (3): 557–65. March 1978. doi:10.1530/acta.0.0870557. PMID 580145.
- ↑ "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. September 2016. doi:10.1016/j.ymgmr.2016.08.006. PMID 27570738.
- ↑ "Metabotypes of congenital adrenal hyperplasia in infants determined by gas chromatography-mass spectrometry in spot urine". J Steroid Biochem Mol Biol 231: 106304. July 2023. doi:10.1016/j.jsbmb.2023.106304. PMID 36990162.
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