Results 171 to 180 of about 1,800 (193)

Physiological basis for an animal model of the renal Fanconi syndrome: use of succinylacetone in the rat

Clinical Science, 1992
1. The biochemical basis for the human renal Fanconi syndrome, including glucosuria, phosphaturia and aminoaciduria, remains enigmatic. This is due, in part, to the lack of an appropriate animal model. Since there is an association between the human genetic disease hereditary tyrosinaemia, for which urinary excretion of the compound succinylacetone ...
P A, Wyss, S B, Boynton, J, Chu, R F, Spencer, K S, Roth   +4 more
openaire   +2 more sources

Plasma succinylacetone is persistently raised after liver transplantation in tyrosinaemia type 1

Journal of Inherited Metabolic Disease, 2012
AbstractBackgroundTyrosinaemia type 1 (HT1) is a rare disorder leading to accumulation of toxic metabolites such as succinylacetone (SA) and a high risk of hepatocellular carcinoma. Children with HT1 traditionally required liver transplantation (OLT) and while the need for this has been reduced by the introduction of nitisinone some still require OLT ...
David C, Bartlett, Mary Anne, Preece, Elisabeth, Holme, Carla, Lloyd, Phil N, Newsome, Patrick J, McKiernan   +5 more
openaire   +2 more sources

Renal heme metabolism in hereditary tyrosinemia: use of succinylacetone in rat renal tubules

Biochimica et Biophysica Acta (BBA) - Biomembranes, 1991
Succinylacetone (SA), a metabolic end-product found in urine from individuals with hereditary tyrosinemia and associated renal Fanconi syndrome and a known inhibitor of hepatic 5-aminolevulinic acid dehydratase (ALAD), has been used to study heme metabolism in isolated rat renal tubules.
P A, Wyss, B E, Carter, S B, Boynton, E, Connor, B, Fowler, K S, Roth   +5 more
openaire   +2 more sources

A patient with urinary succinylacetone‐negative hereditary tyrosinemia type 1

Pediatrics International, 2023
Jun Mori, Taizo Furukawa, Kazuki Kodo, Hisakazu Nakajima, Miori Yuasa, Mitsuru Kubota, Yosuke Shigematsu   +6 more
openaire   +2 more sources

Electrochemical Behavior and Determination of Succinylacetone in Amniotic Fluid

Succinylacetone (SA, 4,6 diketoheptanoic acid) is the primare diagnosis metabolite for hereditable hepatorenal tyrosinemia type I disease. In present researh, voltammetric analysis of succinylacetone was aimed. The optimum conditions for voltammetry were determined. Optimum conditions for voltammetry were determined.
Karacan, Saadet Meral, Yavuz Erdoğan, Behice, Onar, Atiye Nur   +2 more
openaire   +1 more source

Succinylaceton

2018
G. F. Hoffmann, C.-D. Langhans, A. Schulze   +2 more
openaire   +1 more source

Effect of succinylacetone administration on brain heme metabolism and behavior in mice

Biochemical Pharmacology, 1987
K, Kang, L A, Anderson-Burham, J R, Bloomer   +2 more
openaire   +2 more sources

Succinylacetone promising for GVHD

Inpharma Weekly, 1993
openaire   +1 more source

Disruption of Redox Homeostasis by Succinylacetone In Liver and Kidney of Adolescent Rats

Free Radical Biology and Medicine, 2023
Ediandra Tissot Castro, Rafael Teixeira Ribeiro, Júlia Gabrieli Bender, Ângela Beatris Zemniaçak, Sâmela Cunha, Guilhian Leipnitz, Moacir Wajner, Alexandre Umpierrez Amaral   +7 more
openaire   +1 more source

Improved method to determine succinylacetone in dried blood spots for diagnosis of tyrosinemia type 1 using UPLC‐MS/MS

Biomedical Chromatography, 2008
Zuhair Rahbeeni
exaly