Results 161 to 170 of about 1,800 (193)

Detection of succinylacetone and the use of its measurement in mass screening for hereditary tyrosinemia

Clinica Chimica Acta, 1982
A technique designed to measure quantitatively succinylacetone (4,6-dioxoheptanoic acid) is presented. It essentially involves the inhibition of delta-aminolevulinate dehydratase (EC 4.2.1.24) by succinylacetone. Prior to their use in the assay, the samples are heated at 100 degrees C for 30 min in order to transform all succinylacetoacetate (3,5 ...
A, Grenier, A, Lescault, C, Laberge, R, Gagné, O, Mamer   +4 more
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Urinary Succinylacetone Analysis by Gas Chromatography-Mass Spectrometry (GC-MS)

2016
Succinylacetone (SA) is used for the diagnosis and monitoring of patients with tyrosinemia type I (Tyr I). SA is exclusively elevated in blood and urine of patients with Tyr I. As urinary SA concentration is much higher than blood, SA is usually tested in urine samples.
Hongjie, Chen, Chunli, Yu
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On the renal tubular damage in hereditary tyrosinemia and on the formation of succinylacetoacetate and succinylacetone.

Acta paediatrica Scandinavica, 1981
Phenylalanine and homogentisate increase the concentration of succinylacetoacetate and succinylacetone both in serum and urine in patients with hereditary tyrosinemia and therefore increase the excretion of 5-aminolevulinate. Both phenylalanine and homogentisate cause a tubular proteinuria which is in agreement with our hypothesis that their ...
S P, Fällström, B, Lindblad, G, Steen
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Lack of Hemodynamic Effects After Extended Heme Synthesis Inhibition by Succinylacetone in Rats

The Journal of Pharmacology and Experimental Therapeutics, 2010
Hypertyrosinemia (HT) is a life-threatening condition caused in large part by the buildup of tyrosine metabolites and their derivatives. One such metabolite is succinylacetone (SA), a potent irreversible inhibitor of heme biosynthesis. Heme is a key component of numerous enzymes involved in arterial blood pressure (BP) regulation, including nitric ...
Stephane L, Bourque, Carling D, Benjamin, Michael A, Adams, Kanji, Nakatsu   +3 more
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The inclusion of succinylacetone as marker for tyrosinemia type I in expanded newborn screening programs

Rapid Communications in Mass Spectrometry, 2008
Abstract In expanded newborn screening programs by liquid chromatography/tandem mass spectrometry false negatives for tyrosinemia type I are a significant problem. We describe a method for inclusion of succinylacetone in order to avoid false negatives. We studied spots from 13 000 neonates born in Tuscany (January–
LA MARCA, GIANCARLO, Malvagia S, Pasquini E, INNOCENTI, MARZIA, Fernandez MR, Donati MA, ZAMMARCHI, ENRICO   +6 more
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Immunosuppression by succinylacetone. II. Prevention of graft-vs-host disease.

The Journal of Immunology, 1987
Abstract Succinylacetone is a seven-carbon organic ketoacid that we have previously shown to inhibit tumor allograft rejection as well as the primary antibody response to sheep erythrocytes in rats. Because it appeared to be such a potent immunosuppressive agent in our initial studies, we evaluated succinylacetone for its ability to ...
R A, Hess, D P, Tschudy, R M, Blaese
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Evaluation of succinylacetone and nitisinone measurement: analytical performance requirements

2022
SSIEM22-2210Novel diagnostic/laboratory methods including omicsEvaluation of succinylacetone and nitisinone measurement: analytical performance requirementsList of authors:Ridvan Murat Öktem*⠀⠀1, Asli Inci⠀⠀1, Gürsel Biberoglu⠀⠀1, Ilyas Okur⠀⠀1, Fatih Süheyl Ezgü⠀⠀1, Leyla Tümer⠀⠀11 Gazi University, Ankara* = presenting authorBackground: While blood ...
İnci, Aslı, Ezgü, Fatih Süheyl, Biberoğlu, Gürsel, Öktem, Rıdvan Murat, Okur, İlyas, Tümer, Leyla   +5 more
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Augmentation of Hematoporphyrin Uptake and In Vitro-Growth Inhibition of L1210 Leukemia Cells by Succinylacetone

Journal of Urology, 1986
Succinylacetone (SA; 4,6-dioxoheptanoic acid), a specific inhibitor of delta-aminolevulinic acid dehydrase (ALAD) (the second enzyme of the heme biosynthetic pathway), was tested for its effect in L1210 cells from inbred DBA/2 mice. ALAD from broken L1210 cells was completely inhibited by 1 microM SA, but in whole cells activity was decreased only 83 ...
P S, Ebert, R A, Hess, D P, Tschudy
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Detecting Succinylacetone

2007
Mass spectrometry is useful for detecting and measuring a wide variety of metabolites, the presence or amount of which can be indicative of certain conditions or disorders. Thus, mass spectrometry can be used, e.g., to diagnose numerous metabolic disorders associated with altered levels of metabolites.
CERDA B, CHERKASSKIY A, LI Y, LA MARCA, GIANCARLO   +3 more
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Acute depletion of heme by succinylacetone alters vascular responses but does not induce hypertension

Canadian Journal of Physiology and Pharmacology, 2008
Heme plays a critical role in blood pressure regulation because it is required by a number of enzymes that synthesize vascular modulators, including nitric oxide (NO), carbon monoxide (CO), guanosine 3′,5′-cyclic monophosphate (cGMP), endothelium-derived hyperpolarizing factor (EDHF), and prostacyclin. The goal of this study was to examine the vascular
Jonathan, Soong, Michael A, Adams, Kanji, Nakatsu   +2 more
openaire   +2 more sources