Steroids excreted in urine by neonates with 21-hydroxylase deficiency: characterization, using GC-MS and GC-MS/MS, of the D-ring and side chain structure of pregnanes and pregnenes

Steroids
Sofia ChristakoudiN F Taylor

Abstract

Steroid metabolites in urine from neonates with 21-hydroxylase deficiency are predominantly polyhydroxylated 17-hydroxyprogesterone and androgen metabolites, and most have incompletely defined structure. This study forms part of a comprehensive project to characterize and identify these in order to enhance diagnosis and to further elucidate neonatal types of steroid metabolism. Steroids were analyzed, after extraction and enzymatic conjugate hydrolysis, as methyloxime-trimethylsilyl ether derivatives on gas-chromatographs coupled to quadrupole and ion-trap mass-spectrometers. GC-MS and GC-MS/MS spectra, obtained with constant excitation conditions, were used together to determine the structure of the D-ring and the side chain of 20-oxo and 20-hydroxy pregnane(ene)s without oxo groups on the A-, B-, and C-ring. All possible combinations of D-ring and side chain configuration were considered. Most fragmentations could be interpreted as partial or complete D-ring cleavages with loss of the side chain, aided by comparison with spectra of deuterated derivatives and of borohydride reduced metabolites. Possible rearrangement ions are also discussed. More than 140 endogenous metabolites were characterized. GC-MS/MS was especially benef...Continue Reading

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Citations

Nov 30, 2011·Bioanalysis·Lewis CouchmanNorman F Taylor
Dec 18, 2013·Journal of Mass Spectrometry : JMS·Yu-Min JuangChien-Chen Lai
Apr 17, 2015·European Journal of Endocrinology·Angela E TaylorIlpo T Huhtaniemi
Aug 7, 2013·The Journal of Steroid Biochemistry and Molecular Biology·Sofia ChristakoudiNorman F Taylor
Mar 23, 2010·The Journal of Steroid Biochemistry and Molecular Biology·Sofia ChristakoudiNorman F Taylor
May 2, 2016·Clinical Biochemistry·Brian G Keevil
Oct 22, 2014·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Manuel NistalMiguel Reyes-Múgica
May 1, 2014·Expert Review of Endocrinology & Metabolism·Yuhei KoyamaTomonobu Hasegawa
May 26, 2012·Clinical Chemistry·Norman F Taylor, Angel O K Chan
Mar 25, 2021·The Journal of Steroid Biochemistry and Molecular Biology·Julia JunkerChristoph Müller
May 1, 2021·Biomolecules·Michal KaletaMiroslav Strnad

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