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fumarylacetoacetase activity

Known as: 4-fumarylacetoacetate fumarylhydrolase activity, beta-diketonase activity, fumarylacetoacetate hydrolase activity 
Catalysis of the reaction: 4-fumarylacetoacetate + H(2)O = acetoacetate + fumarate + H(+). [EC:3.7.1.2, RHEA:10247]
National Institutes of Health

Papers overview

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1999
1999
Primary pneumococcal peritonitis associated with tyrosinaemia type 1
Tyrosinaemia type 1 is an autosomal recessive disorder characterized by reduced fumarylacetoacetate hydrolase activity (McKusick… 
1990
1990
Hereditary Tyrosinemia of Chronic Course without Rickets and Renal Tubular Dysfunction
ABSTRACT. Three patients with hereditary tyrosinemia type 1, two brothers and one girl, studied at the age of 5, 12 and 15 years… 
1989
1989
[Juvenile form of tyrosinemia type I].
A 1-3/12-year-old Turkish boy born of consanguineous parents was hospitalized in poor general condition with disorientation… 
1988
1988
36 HEREDITARY TYROSINEMIA WITH UNUSUAL PHENOTYPIC EXPRESSION
In the chronic form of tyrosinemia renal tubular dysfunction with secondary hypophosphatemic rickets usually is a major finding… 
1986
1986
The pre- and post-natal diagnosis of tyrosinemia type I and the detection of the carrier state by assay of fumarylacetoacetase.
Fumarylacetoacetase has been determined in fibroblasts, lymphocytes and/or liver tissue from 46 patients affected or presumed to… 
1985
1985
Concentrations of succinylacetone after homogentisate and tyrosine loading in healthy individuals with low fumarylacetoacetase activity.
1981
1981
1183 GLUTATHIONE IS DEFICIENT IN HEREDITARY TYROSINEMIA
Extensive metabolic and enzymatic studies in a patient with hereditary hepatorenal tyrosinemia demonstrated for the first time a… 
1968
1968
Purification of a diketo acid hydrolase from rat liver and its use for the enzymic determination of 3,5-dioxohexanoate (triacetate).
1. A diketo acid hydrolase was purified about 100-fold from rat liver and some of its properties were studied. 2. Comparison of… 
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