Search Results - "Nagel, G T"

Use of determinations of 7-lathosterol (5α-cholest-7-en-3β-ol) and other cholesterol precursors in serum in the study and treatment of disturbances of sterol metabolism, particularly cerebrotendinous xanthomatosis by WOLTHERS, B. G, WALRECHT, H. T, VAN DER MOLEN, J. C, NAGEL, G. T, VAN DOORMAAL, J. J, WIJNANDTS, P. N

“…The sterol composition of sera from patients with cerebrotendinous xanthomatosis (CTX) was investigated by gas chromatographic analysis of saponified extracts,…”
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Phenylketonuria. The in vivo hydroxylation rate of phenylalanine into tyrosine is decreased by van Spronsen, F J, Reijngoud, D J, Smit, G P, Nagel, G T, Stellaard, F, Berger, R, Heymans, H S

“…In phenylketonuria (PKU), the enzyme phenylalanine hydroxylase is deficient, resulting in a decreased conversion of phenylalanine (Phe) into tyrosine (Tyr)…”
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Oral administration of deuterium-labelled polyamines to sucking rat pups: luminal uptake, metabolic fate and effects on gastrointestinal maturation by Dorhout, Bernard, Van Faassen, Anet, Van Beusekom, Christien M., Kingma, Anneke W., De Hoog, Elly, Nagel, Gijs T., Karrenbeld, Arend, Boersma, E. Rudy, Muskiet, Frits A. J.

“…Non-physiological amounts of oral polyamines have been reported to induce precocious gut maturation in rat pups. The aim of the present study was to…”
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Optimization of a classical aromatase activity assay and application in normal, adenomatous and malignant breast parenchyma by Dikkeschei, L.D., Wolthers, B.G., Bos-Zuur, I., de la Rivière, G.Brutel, Nagel, G.T., van der Kolk, D.A., Willemse, P.H.B.

“…The tritium water release assay, originally described for the analysis of aromatase activity in placental tissue, was used to estimate aromatase activity in…”
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Delta-5-androstenediol and its sulphate in serum and urine of normal adults and patients with endocrine diseases by Dikkeschei, L D, Willemse, P H, Wolthers, B G, de Ruyter-Buitenhuis, A W, Nagel, G T

“…We evaluated the role of delta-5-androstenediol (adiol) and its sulphates in health and endocrine diseases. Serum and urine samples from healthy adult men and…”
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New identified 15 beta-hydroxylated 21-deoxy-pregnanes in congenital adrenal hyperplasia due to 21-hydroxylase deficiency by Kraan, G P, Wolthers, B G, van der Molen, J C, Nagel, G T, Drayer, N M, Joannou, G E

“…The identification of 3 new 15 beta-hydroxylated 21-deoxy-pregnanes in the urinary steroid profile of a 4-month-old girl with congenital adrenal hyperplasia…”
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Urinary steroid profile of a newborn suffering from pseudohypoaldosteronism by Wolthers, B.G., Kraan, G.P.B., van der Molen, J.C., Nagel, G.T., Rouwe, C.W., Lenting, F., Boersma, E.R.

“…A case is described of a newborn, admitted to hospital because of severe salt loss at the age of 1 month. Subsequent analysis of urinary steroid excretion, by…”
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The determination of delta-5-androstenediol and its sulphate in serum and urine by gas chromatography-mass spectrometry by Dikkeschei, L D, Wolthers, B G, Willemse, P H, van der Pol, H, de Ruyter-Buitenhuis, A W, Nagel, G T

“…We developed an assay for delta-5-androstenediol (adiol) and delta-5-androstenediol-3-sulphate (adiol-3S) in serum and adiol and total adiol sulphate (adiol-S)…”
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Synthesis and identification of twelve A-ring reduced 6 alpha- and 6 beta-hydroxylated compounds derived from 11-deoxycortisol, corticosterone and 11-dehydrocorticosterone by Kraan, G P, Hartstra, J, Wolthers, B G, van der Molen, J C, Nagel, G T, Drayer, N M, Zijlstra, R W, Kruizinga, W H

“…The synthesis and identification of 12 A-ring reduced 6 alpha-(and 6 beta-)hydroxylated compounds derived from 11-deoxycortisol (S), corticosterone (B) and…”
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Measurement of short-chain acyl-CoA dehydrogenase (SCAD) in cultured skin fibroblasts with hexanoyl-CoA as a competitive inhibitor to eliminate the contribution of medium-chain acyl-CoA dehydrogenase by Niezen-Koning, K E, Wanders, R J, Nagel, G T, Sewell, A C, Heymans, H S

“…Short-chain acyl-CoA dehydrogenase (SCAD) deficiency has so far been reported in only very few patients. This is due, in part, to the problems involved in…”
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Detection of 3 beta-hydroxysteroid dehydrogenase deficiency in a newborn by means of urinary steroid analysis by Wolthers, B G, de Vries, I J, Volmer, M, Nagel, G T

“…A urinary steroid excretion pattern of a 3-wk-old newborn, suffering from 3 beta-hydroxysteroid dehydrogenase (3 beta-HSD) deficiency, has been produced,…”
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GC-MS determination of ratios of stable-isotope labelled to natural urea using [13C15N2]urea for studying urea kinetics in serum and as a means to validate routine methods for the quantitative assay of urea in dialysate by Wolthers, B G, Tepper, T, Withag, A, Nagel, G T, de Haan, T H, van Leeuwen, J J, Stegeman, C A, Huisman, R M

“…A GC-MS determination of urea in serum or spent dialysate is described, using 13C15N2-labelled urea and assaying the area ratio of labelled to natural urea by…”
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Determination of cholic acid and chenodeoxycholic acid pool sizes and fractional turnover rates by means of stable isotope dilution technique, making use of deuterated cholic acid and chenodeoxycholic acid by Koopman, B J, Kuipers, F, Bijleveld, C M, van der Molen, J C, Nagel, G T, Vonk, R J, Wolthers, B G

“…A procedure is described for the simultaneous determination of cholic acid and chenodeoxycholic acid pool sizes and fractional turnover rates. After oral…”
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Capillary gas chromatographic determinations of urinary bile acids and bile alcohols in CTX patients proving the ineffectivity of ursodeoxycholic acid treatment by Koopman, B J, Wolthers, B G, van der Molen, J C, Nagel, G T, Waterreus, R J, Oosterhuis, H J

“…Urine samples and serum samples of a patient with cerebrotendinous xanthomatosis (CTX) were investigated by means of capillary gas chromatography, both before…”
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Specific and sensitive determination of medroxyprogesterone acetate in human serum by gas chromatography-mass spectrometry by Dikkeschei, L D, van Veelen, H, Nagel, G T, Willemse, P H, Wolthers, B G

“…The mass fragmentographic determination of medroxyprogesterone acetate (MPA) in serum, using as internal standard medroxyprogesterone propionate (MPP)…”
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A new, simple assay for long-chain acyl-CoA dehydrogenase in cultured skin fibroblasts using stable isotopes and GC-MS by Niezen-Koning, K E, Wanders, R J, Nagel, G T, IJlst, L, Heymans, H S

“…In this paper, we present a new method for measurement of long-chain acyl-CoA dehydrogenase (LCAD) activities in cultured skin fibroblasts. The method is based…”
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Increased (23R)-hydroxylase activity in patients suffering from cerebrotendinous xanthomatosis, resulting in (23R)-hydroxylation of bile acids by Koopman, B J, Wolthers, B G, Van der Molen, J C, Nagel, G T, Rutgers, H, Strijtveen, B, Kaptein, B

“…Patients suffering from cerebrotendinous xanthomatosis, an inborn error of metabolism in bile acid synthesis, excrete excessive amounts of 23-hydroxylated bile…”
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Abnormal urinary bile acids in a patient suffering from cerebrotendinous xanthomatosis during oral administration of ursodeoxycholic acid by Koopman, B J, Wolthers, B G, van der Molen, J C, Nagel, G T, Kruizinga, W

“…The urinary bile acid profile, obtained by capillary gas chromatography, of a patient suffering from cerebrotendinous xanthomatosis and treated with…”
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Catabolism of polyamines in the rat. Polyamines and their non-alpha-amino acid metabolites by Van den Berg, G A, Elzinga, H, Nagel, G T, Kingma, A W, Muskiet, F A

“…The metabolic fate of stable isotopically labeled polyamines was investigated after their first and second intraperitoneal injection in rats. Using gas…”
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Determination of the urinary cortisol production rate using (1,2,3,4-13C)cortisol. Isotope dilution analyses at very small enrichments by Chapman, T E, Kraan, G P, Drayer, N M, Nagel, G T, Wolthers, B G

“…An isotope dilution mass spectrometric method to determine the urinary cortisol production rate (CPR) in babies and children is described. The method uses…”
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